A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.
A term used to describe a variety of localized asymmetrical SKIN thickening that is similar to those of SYSTEMIC SCLERODERMA but without the disease features in the multiple internal organs and BLOOD VESSELS. Lesions may be characterized as patches or plaques (morphea), bands (linear), or nodules.
The age, developmental stage, or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual.
A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability.
The least progressive form of SYSTEMIC SCLERODERMA with skin thickening restricted to the face, neck and areas distal to the elbows and/or knees, sparing the trunk. The CREST SYNDROME is a form of limited scleroderma.
The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
An autoimmune disorder mainly affecting young adults and characterized by destruction of myelin in the central nervous system. Pathologic findings include multiple sharply demarcated areas of demyelination throughout the white matter of the central nervous system. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia, and bladder dysfunction. The usual pattern is one of recurrent attacks followed by partial recovery (see MULTIPLE SCLEROSIS, RELAPSING-REMITTING), but acute fulminating and chronic progressive forms (see MULTIPLE SCLEROSIS, CHRONIC PROGRESSIVE) also occur. (Adams et al., Principles of Neurology, 6th ed, p903)
An idiopathic vascular disorder characterized by bilateral Raynaud phenomenon, the abrupt onset of digital paleness or CYANOSIS in response to cold exposure or stress.
A mild form of LIMITED SCLERODERMA, a multi-system disorder. Its features include symptoms of CALCINOSIS; RAYNAUD DISEASE; ESOPHAGEAL MOTILITY DISORDERS; sclerodactyly, and TELANGIECTASIS. When the defect in esophageal function is not prominent, it is known as CRST syndrome.
Permanent dilation of preexisting blood vessels (CAPILLARIES; ARTERIOLES; VENULES) creating small focal red lesions, most commonly in the skin or mucous membranes. It is characterized by the prominence of skin blood vessels, such as vascular spiders.
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
The transference of either one or both of the lungs from one human or animal to another.
Glomerulonephritis associated with autoimmune disease SYSTEMIC LUPUS ERYTHEMATOSUS. Lupus nephritis is histologically classified into 6 classes: class I - normal glomeruli, class II - pure mesangial alterations, class III - focal segmental glomerulonephritis, class IV - diffuse glomerulonephritis, class V - diffuse membranous glomerulonephritis, and class VI - advanced sclerosing glomerulonephritis (The World Health Organization classification 1982).
Conduct and synthesis of systematic research comparing interventions and strategies to prevent, diagnose, treat, and monitor health conditions. The purpose of this research is to inform patients, providers, and decision-makers, responding to their expressed needs, about which interventions are most effective for which patients under specific circumstances. (hhs.gov/recovery/programs/cer/draftdefinition.html accessed 6/12/2009)
Disturbances in the ACID-BASE EQUILIBRIUM of the body.
A plant genus of the family ORCHIDACEAE that is the source of the familiar flavoring used in foods and medicines (FLAVORING AGENTS).
The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.
Disorders of connective tissue, especially the joints and related structures, characterized by inflammation, degeneration, or metabolic derangement.
A condition of low alertness or cognitive impairment, usually associated with prolonged mental activities or stress.
A state arrived at through prolonged and strong contraction of a muscle. Studies in athletes during prolonged submaximal exercise have shown that muscle fatigue increases in almost direct proportion to the rate of muscle glycogen depletion. Muscle fatigue in short-term maximal exercise is associated with oxygen lack and an increased level of blood and muscle lactic acid, and an accompanying increase in hydrogen-ion concentration in the exercised muscle.
A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.
The concept covering the physical and mental conditions of men.
A process in which normal lung tissues are progressively replaced by FIBROBLASTS and COLLAGEN causing an irreversible loss of the ability to transfer oxygen into the bloodstream via PULMONARY ALVEOLI. Patients show progressive DYSPNEA finally resulting in death.
A common interstitial lung disease of unknown etiology, usually occurring between 50-70 years of age. Clinically, it is characterized by an insidious onset of breathlessness with exertion and a nonproductive cough, leading to progressive DYSPNEA. Pathological features show scant interstitial inflammation, patchy collagen fibrosis, prominent fibroblast proliferation foci, and microscopic honeycomb change.
Substernal pain or burning sensation, usually associated with regurgitation of gastric juice into the esophagus.

Autoantibodies to RNA polymerases recognize multiple subunits and demonstrate cross-reactivity with RNA polymerase complexes. (1/2023)

OBJECTIVE: To determine the subunit specificity of autoantibody directed to RNA polymerases (RNAP) I, II, and III, which is one of the major autoantibody responses in patients with systemic sclerosis (SSc). METHODS: Thirty-two SSc sera with anti-RNAP antibodies (23 with anti-RNAP I/III, 5 with anti-RNAP I/III and II, and 4 with anti-RNAP II alone) were analyzed by immunoblotting using affinity-purified RNAP and by immunoprecipitation using 35S-labeled cell extracts in which RNAP complexes were dissociated. Antibodies bound to individual RNAP subunits were eluted from preparative immunoblots and were further analyzed by immunoblotting and immunoprecipitation. RESULTS: At least 15 different proteins were bound by antibodies in anti-RNAP-positive SSc sera in various combinations. All 9 sera immunoprecipitating RNAP II and all 28 sera immunoprecipitating RNAP I/III recognized the large subunit proteins of RNAP II and III, respectively. Reactivity to RNAP I large subunits was strongly associated with bright nucleolar staining by indirect immunofluorescence. Affinity-purified antibodies that recognized a 62-kd subunit protein cross-reacted with a 43-kd subunit protein and immunoprecipitated both RNAP I and RNAP III. Antibodies that recognized a 21-kd subunit protein obtained from sera that were positive for anti-RNAP I/III and II antibodies immunoprecipitated both RNAP II and RNAP III. CONCLUSION: Anti-RNAP antibodies recognize multiple subunits of RNAP I, II, and III. Moreover, the results of this study provide the first direct evidence that antibodies that recognize shared subunits of human RNAPs or epitopes present on different human RNAP subunits are responsible for the recognition of multiple RNAPs by SSc sera.  (+info)

Interferon-alpha does not improve outcome at one year in patients with diffuse cutaneous scleroderma: results of a randomized, double-blind, placebo-controlled trial. (2/2023)

OBJECTIVE: To determine whether interferon-alpha (IFNalpha) reduces the severity of skin involvement in early (<3 years) diffuse scleroderma. METHODS: In a randomized, placebo-controlled, double-blind trial, 35 patients with early scleroderma received subcutaneous injections of either IFNalpha (13.5 x 10(6) units per week in divided doses) or indistinguishable placebo. Outcomes assessed were the modified Rodnan skin score, as determined by a single observer at baseline, 6 months, and 12 months, as well as data on renal, cardiac, and lung function. Pre- and posttreatment skin biopsy samples were analyzed and blood was obtained for assessment of procollagen peptide levels. RESULTS: There were 11 withdrawals from the IFNalpha group and 3 from the placebo group due to either toxicity, lack of efficacy, or death. In the intent-to-treat analysis, there was a greater improvement in the skin score in the placebo group between 0 and 12 months (mean change IFNalpha -4.7 versus placebo -7.5; P = 0.36). There was also a greater deterioration in lung function in patients receiving active therapy, as assessed by either the forced vital capacity (mean change IFNalpha -8.2 versus placebo +1.3; P = 0.01) or the diffusing capacity for carbon monoxide (mean change IFNalpha -9.3 versus placebo +4.7; P = 0.002). Skin biopsy showed no significant decrease in collagen synthesis in the IFNalpha group, and no significant differences in the levels of procollagen peptides were seen between the 2 groups. CONCLUSION: This study suggests that IFNalpha is of no value in the treatment of scleroderma, and that it may in fact be deleterious.  (+info)

Long-term fetal microchimerism in peripheral blood mononuclear cell subsets in healthy women and women with scleroderma. (3/2023)

Fetal CD34(+) CD38(+) cells have recently been found to persist in maternal peripheral blood for many years after pregnancy. CD34(+) CD38(+) cells are progenitor cells that can differentiate into mature immune-competent cells. We asked whether long-term fetal microchimerism occurs in T lymphocyte, B lymphocyte, monocyte, and natural-killer cell populations of previously pregnant women. We targeted women with sons and used polymerase chain reaction for a Y-chromosome-specific sequence to test DNA extracted from peripheral blood mononuclear cells (PBMC) and from CD3, CD19, CD14, and CD56/16 sorted subsets. We also asked whether persistent microchimerism might contribute to subsequent autoimmune disease in the mother and included women with the autoimmune disease scleroderma. Scleroderma has a peak incidence in women after childbearing years and has clinical similarities to chronic graft-versus-host disease that occurs after allogeneic hematopoietic stem-cell transplantation, known to involve chimerism. Sixty-eight parous women were studied for male DNA in PBMC and 20 for PBMC subsets. Microchimerism was found in PBMC from 33% (16 of 48) of healthy women and 60% (12 of 20) women with scleroderma, P =.046. Microchimerism was found in some women in CD3, CD19, CD14, and CD56/16 subsets including up to 38 years after pregnancy. Microchimerism in PBMC subsets was not appreciably more frequent in scleroderma patients than in healthy controls. Overall, microchimerism was found in CD3, CD19, and CD14 subsets in approximately one third of women and in CD56/16 in one half of women. HLA typing of mothers and sons indicated that HLA compatibility was not a requirement for persistent microchimerism in PBMC subsets. Fetal microchimerism in the face of HLA disparity implies that specific maternal immunoregulatory pathways exist that permit persistence but prevent effector function of these cells in normal women. Although microchimerism in PBMC was more frequent in women with scleroderma than healthy controls additional studies will be necessary to determine whether microchimerism plays a role in the pathogenesis of this or other autoimmune diseases.  (+info)

Evidence of cell-mediated cardiac myocyte injury involved in the heart failure of a patient with progressive systemic sclerosis. (4/2023)

A 54-year-old woman with progressive systemic sclerosis (PSS) was admitted to hospital because of dyspnea and chest pain. Echocardiogram revealed diffuse hypokinesis of the left ventricle (ejection fraction 24%). Methylprednisolone, heparin, and diuretics were administered, without benefit. Anemia, thrombocytopenia, and renal dysfunction rapidly progressed, and she died of heart failure on the 14th hospital day. Immunohistochemical study of the myocardial tissue showed mild to moderate cell infiltration, mainly consisting of natural killer (NK) cells, macrophages, cytotoxic T lymphocytes (CTLs), and T helper cells. Perforin, a cytolytic factor, was expressed in the infiltrating CTLs and NK cells, indicating that these cells were activated killer cells. Furthermore, human leukocyte antigen classes I and II, intercellular adhesion molecule-1, as well as costimulatory molecules B7-1, B7-2, and CD40, all of which are known not to be expressed in cardiac myocytes under normal conditions, were moderately to strongly expressed in cardiac myocytes. There was no detectable level of enterovirus genomes in the polymerase chain reaction products from the myocardial tissue of this patient. These findings strongly suggest that the infiltrating killer cells recognized cardiac myocytes as target cells and directly damaged them by releasing perforin. Enhanced expression of these antigens may have played an important role in the activation and cytotoxicity of the infiltrating killer cells. Absence of enterovirus genomes in the myocardial tissue may suggest that this autoimmune process is primarily induced by PSS.  (+info)

Influence of ethnic background on clinical and serologic features in patients with systemic sclerosis and anti-DNA topoisomerase I antibody. (5/2023)

OBJECTIVE: To investigate the effect of ethnicity on clinical and serologic expression in patients with systemic sclerosis (SSc) and anti-DNA topoisomerase I (anti-topo I) antibody. METHODS: Clinical and serologic features, as well as HLA class II allele frequencies, were compared among 47 North American white, 15 North American black, 43 Japanese, and 12 Choctaw Native American SSc patients with anti-topo I antibody. RESULTS: The frequency of progressive pulmonary interstitial fibrosis was lower, and cumulative survival rates were better in white compared with black and Japanese patients. Sera of white and black patients frequently recognized the portion adjacent to the carboxyl terminus of topo I, sera of Japanese patients preferentially recognized the portion adjacent to the amino terminus of topo I, and sera of Choctaw patients recognized both portions of topo I. Anti-RNA polymerase II and anti-SSA/Ro antibodies were present together with anti-topo I antibody more frequently in sera of Japanese patients than in sera of white patients. The HLA-DRB1 alleles associated with anti-topo I antibody differed; i.e., DRB1*1101-*1104 in whites and blacks, DRB1*1502 in Japanese, and DRB1*1602 in Choctaws. Multivariate analysis showed that ethnic background was an independent determinant affecting development of severe lung disease as well as survival. CONCLUSION: Clinical and serologic features in SSc patients were strongly influenced by ethnic background. The variability of disease expression in the 4 ethnic groups suggests that multiple factors linked to ethnicity, including genetic and environmental factors, modulate clinical manifestations, disease course, and autoantibody status in SSc.  (+info)

Pulmonary artery pressure variation in patients with connective tissue disease: 24 hour ambulatory pulmonary artery pressure monitoring. (6/2023)

BACKGROUND: The specific contribution of secondary pulmonary hypertension to the morbidity and mortality of patients with underlying lung disease can be difficult to assess from single measurements of pulmonary artery pressure. We have studied patients with secondary pulmonary hypertension using an ambulatory system for measuring continuous pulmonary artery pressure (PAP). We chose to study patients with connective tissue disease because they represent a group at high risk of pulmonary vascular disease, but with little disturbance of lung function. METHODS: Six patients (five with progressive systemic sclerosis and one with systemic lupus erythematosis) were studied. They underwent preliminary cardiopulmonary investigations followed by Doppler echocardiography, right heart catheterisation, and ambulatory pulmonary artery pressure monitoring to measure changes in pressure over a 24 hour period including during a formal exercise test. RESULTS: All patients had pulmonary hypertension as measured by Doppler echocardiography with estimated pulmonary artery systolic pressures of 40-100 mm Hg. Pulmonary function testing revealed virtually normal spirometric values (mean FEV1 86.9% predicted) but marked reduction in CO gas transfer factor (KCO 57.8% predicted). Exercise responses were impaired with mean VO2max 50.6% predicted. Ambulatory PAP monitoring indicated significant changes in pressures with variation in posture and activity throughout 24 hours. Resting PAP did not predict the change in PAP seen on exercise. CONCLUSION: Conventional methods of assessment of the pulmonary circulation based on single measurements in the supine position may underestimate the stresses faced by the right side of the circulation. This ambulatory system allows monitoring of pulmonary haemodynamics continuously over 24 hours during normal activities of daily living. These measurements may increase our understanding of the contribution made by secondary pulmonary hypertension to the morbidity and mortality of the underlying lung disease.  (+info)

Animal model of sclerotic skin. I: Local injections of bleomycin induce sclerotic skin mimicking scleroderma. (7/2023)

We have established a mouse model for scleroderma induced by repeated local injections of bleomycin (BLM). Daily injection of BLM at a dose of >10 microg per ml for 4 wk induced histologic changes of dermal sclerosis, but not fibrosis, with thickened and homogenous collagen bundles and cellular infiltrates in BALB/C mice, whereas clinical signs of scleroderma were not apparent. In addition, lung fibrosis was also induced preceding the cutaneous changes. Sclerotic changes were not found in other sites of the skin distant from the injection site. Dermal sclerosis could also be induced by injecting BLM only every other day. The sclerotic changes of the dermis were sustained after ceasing BLM applications for at least 6 wk. Mast cells gradually increased in number as the sclerotic changes developed. Marked degranulation of mast cells was observed with elevated histamine release. The amount of hydroxyproline in skin was significantly increased at 4 wk of BLM treatment as compared with that in untreated or phosphate-buffered saline-treated mice. Anti-nuclear antibody was detected in serum of BLM-treated mice. Transforming growth factor-beta1 mRNA was detected at an early phase, while transforming growth factor-beta2 mRNA was strongly expressed at 4 wk when the sclerotic features were prominent. These results suggest that dermal sclerosis induced by BLM closely resembles systemic sclerosis both histologically and biochemically. Our mouse model can provide a powerful tool of inducing dermal sclerosis to examine the pathogenesis and the therapeutic approach of scleroderma.  (+info)

A potential role for protease nexin 1 overexpression in the pathogenesis of scleroderma. (8/2023)

Scleroderma currently affects approximately 75,000-100,000 individuals in the United States. Fibroblasts isolated from lesional skin of scleroderma patients overexpress collagens and other matrix components, and this abnormality is maintained for multiple passages in culture. To understand the molecular basis for matrix gene overexpression, we performed a differential display comparison of fibroblasts from clinically lesional and nonlesional scleroderma skin. The results suggested that protease nexin 1 (PN1), a protease inhibitor, is overexpressed in scleroderma fibroblasts. Northern blot verification showed that lesional and nonlesional scleroderma fibroblasts had three- to five-fold increased levels of PN1 mRNA compared with healthy fibroblasts. Western analysis showed that scleroderma fibroblasts also secreted more PN1. In situ hybridization of skin biopsy specimens demonstrated PN1 expression in the dermis of four out of six scleroderma patients but no PN1 expression in the dermis of six healthy volunteers. Transient or stable overexpression of PN1 in mouse 3T3 fibroblasts increased collagen promoter activity or endogenous collagen transcript levels, respectively. PN1 mutagenized at its active site and antisense PN1 both failed to increase collagen promoter activity. These results suggest that overexpression of enzymatically active PN1 may play a pathogenic role in the development of the scleroderma phenotype.  (+info)

Looking for Systemic scleroderma? Find out information about Systemic scleroderma. An abnormal increase in collagenous connective tissue in the skin. Also known as chorionitis; dermatosclerosis; scleriasis. a disease of man, of the group... Explanation of Systemic scleroderma
Diffuse Systemic scleroderma with severe pulmonary fibrosis. April 2008. My name is Kristine. I use my nickname Kramstine. Kram means hug in Danish which is the language we speak in my country Denmark. I am 31 years old. Here is my history before AP:. In the autumn 2001 I was diagnosed with Diffuse Systemic Scleroderma[/b]. My symptoms started the same year in spring. First I felt heartburn for no good reason and started waking up with stiff, swollen hands/fingers in the morning (this continued most of the day, and I also developed raynauds syndrome[/b]). This was what made me go to the doctor at first. He didn?t have much to say, but when all my joints (knees, wrists, elboes?) started hurting and I couldn?t walk the stairs without pain, I had another doctor check me. He did some blood tests that showed I had arthritis. From there till the final conclusion, that my disease is scleroderma, there were a lot of testing and a lot of waiting. In the meanwhile I had had a cough that wouldn?t go away ...
Background/Purpose: Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. Longitudinal prospective follow up data of patients with jSSc is rare. In the international juvenile systemic scleroderma cohort (jSScC) patients are followed with a standardized assessment prospectively.. Methods: Patients diagnosed according the ACR 2013 criteria for systemic sclerosis were included, if they developed the first non-Raynaud symptom before the age of 16 and were under the age of 18 at the time of inclusion. Patients were followed prospectively every 6 months with a standardized assessment.. Results: 39 patients in the JSScC had 36 months follow up. 80% had a diffuse subtype. 95% of the patients were Caucasian origin and 80% female. Mean disease duration at time of inclusion was 3.5 years. Mean age onset of Raynauds was 8.8 years and mean age of onset at the first non-Raynaud´s was 9.5 years. The MRSS dropped from the time point of the inclusion into the ...
Systemic scleroderma is a connective tissue disorder, which affects the skin, vessels and organs such as the heart, digestive tract or lungs.… Systemic Scleroderma: Read more about Symptoms, Diagnosis, Treatment, Complications, Causes and Prognosis.
Tweet Today I would like to share with you a report on a study of the effects of manual lymph drainage (Vodder technique) on systemic scleroderma, which was published in the January 10 issue of Lymphologie Aktuell, the member journal of the German Society of Lymphology.. Introduction:. The impacts on the quality of life . . . → Read More: Effects of Manual Lymph Drainage on Systemic Scleroderma. ...
Tweet Today I would like to share with you a report on a study of the effects of manual lymph drainage (Vodder technique) on systemic scleroderma, which was published in the January 10 issue of Lymphologie Aktuell, the member journal of the German Society of Lymphology.. Introduction:. The impacts on the quality of life . . . → Read More: Effects of Manual Lymph Drainage on Systemic Scleroderma. ...
Not everyone with scleroderma develops this degree of skin hardening. However, it is this symptom that has earned scleroderma the nickname of the disease that turns people to stone.. Systemic scleroderma often affects the hands. The initial stage is swelling (edema), which can last for weeks, months, or years. Often the swelling is intermittent and worse in the morning. It can cause the fingers to look like sausages, with far fewer wrinkles. Skin tightness in the hands can make it impossible to pinch the skin on the fingers ...
These are photos taken after a digital sympathectomy, in a systemic scleroderma patient, from the International Scleroderma Networks Photo Repository.
Read about how different drugs are being tested in order to have a better prognosis for PAH, the risk of which is highest in people with systemic scleroderma.
Systemic scleroderma in children is very rare and is considered similar to adult-onset disease. In adults, new etiopathogenetic and therapeutic approaches have emerged in recent years. For instance, i
Read about the findings of a new study claiming that epigenetic changes may be involved in the development of systemic scleroderma.
To the Editors: We read with great interest the review by Steen and colleagues (1) on the outcome of scleroderma renal crisis before and after the availability of angiotensin converting enzyme (ACE) inhibitors. Of the 55 patients in the study who had scleroderma renal crisis, the continuation or acceleration of renal insufficiency in 4 patients raised the question of captopril-related renal toxicity. The authors concluded that Captopril could not have caused renal deterioration in these patients because none of the 4 patients had peripheral blood or urine eosinophilia or improvement of renal failure after withdrawal of captopril.. We wish to ...
See If You Qualify for a Local Scleroderma Research Study!. Owens, like many others, may not have been aware of the many ways systemic scleroderma can affect the bodys organs. With scleroderma affecting an estimated 300,000 Americans and African Americans being diagnosed more frequently and at an earlier age than other ethnic groups, its important to know the facts.. Myth: Scleroderma is a disease that only affects the skin.. Truth: Scleroderma is primarily characterized by thickening of the skin, but this chronic connective tissue disease can affect the blood vessels and internal organs in addition to the skin. Localized scleroderma affects certain parts of the body (usually the skin), but with systemic scleroderma the entire body can be affected - skin, kidneys, digestion, joint, teeth, lungs, heart and Raynauds Phenomenon.. Myth: Scleroderma is a genetic disease.. Truth: Scientists do not know what causes scleroderma, but researchers do not believe it is passed on through genes. According ...
TY - JOUR. T1 - Therapies for scleroderma-related pulmonary arterial hypertension. AU - Hassoun, Paul M.. PY - 2009/9/14. Y1 - 2009/9/14. N2 - Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer from it. Indeed, response to modern medical therapy has been disappointing in scleroderma-related PAH compared with other forms of PAH from the WHO group 1 classification of diseases, despite similar histological changes involving the pulmonary vasculature. This review discusses specific features of scleroderma-related PAH, currently available and US FDA-approved therapy for this syndrome, as well as potential future therapeutic developments based on newly acquired knowledge of this disorder.. AB - Pulmonary arterial hypertension (PAH), a common complication of systemic sclerosis, carries a very severe prognosis and is one of the leading causes of death in patients who suffer ...
Systemic sclerosis (SSc) is a connective tissue disease characterized by excessive collagen deposition, autoimmunity and by vascular hyper-reactivity and obliterative microvascular phenomena that involves multiple organs. Scleroderma Renal Crisis (SRC) occurs in 5% of patients and mainly with diffuse cutaneous SSc. The routine use of angiotensin-converting enzyme inhibitors (ACEI) has been reported to dramatically improve outcome, with a fall of the 12-month mortality from 76% to less than 15% in the United-States. Despite prognostic improvement, SRC remains a severe manifestation of SSc and functional outcome and survival remains poor. Bosentan is a specific, orally active, dual endothelin receptor antagonist that has recently been approved for the treatment of primary pulmonary arterial hypertension and for the prevention of ischemic digital ulcers. Bosentan could have therapeutic benefits on others vascular injuries and particularly in SRC ...
This prospective, double-blind, placebo-controlled, multi-center, randomized trial will evaluate the effect of rituximab on disease progression in subjects with SSc-PAH receiving concurrent stable-dose standard medical therapy with a prostanoid, endothelin receptor antagonist, and/or phosphodiesterase 5 (PDE-5) inhibitor. The study will focus on assessment of clinical response and safety measures longitudinally. In addition, the effects of treatment with rituximab on the underlying immune mechanisms associated with B-cell dysregulation and pathogenic autoantibody response in this disease will be investigated. 1000 mg of rituximab or placebo will be administered as two IV infusions given two weeks apart. Clinical assessments and sample collection will occur at monthly visits through Week 48. If a participant has not recovered B cells by Week 48, B cell studies will be conducted quarterly until reconstitution is documented or for 2 years after initial treatment.. This trial will include a ...
A patient with scleroderma renal crisis is described. At presentation he had severe hypertension, deteriorating renal function, microangiopathic haemolytic anaemia, and elevated levels of renin, aldosterone and noradrenaline. Enalapril controlled blood pressure, stabilized renal function, lowered aldosterone and noradrenaline levels, and improved peripheral circulation. It appears that converting-enzyme inhibitors can favourably alter the outlook of this otherwise fatal disorder.. ...
This animated video from Demystifying Medicine explains the complication of pulmonary fibrosis in scleroderma patients. It begins by sharing that 80 percent of Asian scleroderma patients suffer from some form of pulmonary disease and that its the leading cause of death among this patient population.. MORE: When to consider professional help while dealing with PF. The film shows a picture of a lung which has undergone serious fibrosis and explains that between 30 percent and 70 percent of Asian scleroderma patients suffer from pulmonary fibrosis. It goes on to describe the various other causes of pulmonary fibrosis, the symptoms of the disease and some of the treatment options available to scleroderma patients including pulmonary rehabilitation, medications, and lung transplant.. MORE: How to help others deal with your disease. Lung Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended ...
Systemic sclerosis (SSc) is a clinically heterogenous chronic fibrotic disease which affects skin and internal organs. While the pathogenesis of SSc remains unknown, the hallmark of both localized and diffuse SSc in the skin is the replacement of normal dermal architecture with excessive deposition of collagen and other connective tissue macromolecules. Progressive replacement of tissue architecture by collagen-rich extracellular matrix (ECM) results in functional impairment of affected organs. Fibrotic damage to these affected organs accounts for much of the morbidity and mortality concomitant with SSc, particularly in the lungs. Myofibroblasts are the primary ECM-secreting cells during wound healing and fibrosis. Myocardin-related transcription factor A (MRTF-A), is an important regulator of myofibroblast differentiation, depending on serum response factor (SRF) for smooth muscle actin (SMA) and Sp1 in the regulation of collagen gene expression. MRTF-A continually shuttles between the nucleus ...
Result: Of 608 SSc patients seen during the study period, 19 SRC cases were identified, resulting in an SRC prevalence of 3.13%, with 76 matched controls. Of the 19 cases, mean ± SD age and median (interquartile range 1-3) disease duration was 56.2 ± 13.8 years and 5 (3-22) months, respectively. Seventeen patients (89.5%) had diffuse cutaneous SSc. Twelve patients (63.2%) had hypertensive renal crisis and seven (36.8%) had normotensive renal crisis. Multivariate conditional logistic regression analyses showed that digital gangrene (adjusted odd ratio [AOR] 31.41, 95% CI = 1.16-852.23, P = 0.041), current prednisolone dose ≥ 15 mg/day (AOR 31.22, 95% CI = 1.59-613.85, P = 0.024), serum albumin < 3 mg/dL (AOR 7.97, 95% CI = 1.49-42.56, P = 0.015), and cardiac involvement (AOR = 6.62, 95% CI = 1.08-40.63, P = 0.041) were independent risk factors for SRC. Fifteen SRC patients (78.9%) required dialysis and 10 (52.6%) died ...
Actemra (tocilizumab) is a treatment by Genentech approved for the treatment of systemic sclerosis-associated interstitial lung disease.
Roughly 300,000 people in the United States suffer from scleroderma. This chronic connective tissue disease results from an over-production of collagen in the skin and other organs. Scleroderma usually appears in people between the ages of 25 and 55. Women get scleroderma more often than men. The disease worsens slowly over years.. There are two types of scleroderma: localized scleroderma, which involves only the skin, and systemic scleroderma, which involves the skin and other organs, such as the heart, lungs, kidneys, intestine and gallbladder. Typical symptoms of the skin include skin hardening, skin that is abnormally dark or light, skin thickening, shiny hands and forearms, small white lumps beneath the skins surface, tight facial skin, ulcerations on the fingers or toes and change in color of the fingers and toes from exposure to heat or cold. Other symptoms impact bones, muscles, lungs and the digestive tract.. There is no known cause of scleroderma, nor is there a cure. There are ...
Scleroderma - a progressive disease with a characteristic change in the skin, musculoskeletal system and internal organs. The disease is an inflammatory lesion of small vessels of the body. Perhaps, the disease has a genetic predisposition. However, significantly provoking factors of its occurrence are such external hazards like hypothermia, vibration in the workplace, transferred infection of the nervous system. The development of inflammation of small blood vessels leads to the widening of collagen around them, and fibrous tissue, as well as specific changes in their walls - a thickening, loss of elasticity, perhaps even the complete closure of the lumen of small vessels. These changes, in turn, lead to impaired blood supply to all organs and tissues involved in the pathological process. Inadequate blood supply to the tissues leads to thinning (eg, esophageal and stomach), or, alternatively, thickening (the wall of the alveoli in the lungs), a violation of their basic functions (absorption in ...
Pams Story: Scleroderma. My story began 1972. This part is relevant to my diagnosis. Five months after my marriage breakdown, while I was at work, my ex-husband came and took my seven-year-old son from the baby sitter.
Boehringer Ingelheims Nintedanib Receives CHMPs Positive Opinion to Treat Systemic Sclerosis-Associated Interstitial Lung Disease
Distler, Oliver; Brown, Kevin K; Distler, Jörg H W; Assassi, Shervin; Maher, Toby M; Cottin, Vincent; Varga, John; Coeck, Carl; Gahlemann, Martina; Sauter, Wiebke; Schmidt, Hendrik; Highland, Kristin B; SENSCIS™ trial investigators (2017). Design of a randomised, placebo-controlled clinical trial of nintedanib in patients with systemic sclerosis-associated interstitial lung disease (SENSCIS™). Clinical and Experimental Rheumatology, 35 Suppl(4):75-81. ...
Thank you for visiting my personal fundraising page to support the Stepping Out to Cure Scleroderma event!. Donating through this site is simple, fast and totally secure. It is also the most efficient way to make a contribution to support me and help raise funds for scleroderma patients. To make a donation, click the Donate Now button that appears under my photo. Then, just follow the instructions.. If you would like to join me at the event, and walk and help raise funds, please click on Join My Team and follow the directions.. Please help today! I sincerely appreciate your support, and please share this with anyone who you think might want to help.. Help me bring more awareness to scleroderma so we can find a cure!. Thank you!. Love, Linda. Diagnosed with Systemic Scleroderma w/complications 2014. ...
Anti-Topoisomerase I antibody - ChIP Grade (ab3825) has been cited in 19 publications. References for Human, Mouse in ChIP, ICC/IF, IP, WB
Systemic sclerosis (SSc) is an autoimmune disease with a heterogeneous range of skin and internal organ involvement, a progressive course, incompletely understood pathogenesis, and unpredictable outcome. The disease is most known for varying degrees of fibrosis of the skin. Of great concern for patient prognosis in SSc is internal organ involvement, and improvements in monitoring internal organ involvement are needed. Most treatments for SSc target specific symptoms and/or internal organ complications, and show only modest efficacy at improving patient outcome. SSc patients are classified into one of two groups, diffuse (dSSc) or limited (lSSc). Further sub-dividing patients into groups beyond those currently recognized may direct drug treatments and improve determination of prognosis. A focus of the research presented here was to determine through genome-wide gene expression analysis of dSSc skin if subsets of patients were consistently detectible and how these subsets would change over time. ...
Systemic sclerosis (SSc) is an autoimmune disease with a heterogeneous range of skin and internal organ involvement, a progressive course, incompletely understood pathogenesis, and unpredictable outcome. The disease is most known for varying degrees of fibrosis of the skin. Of great concern for patient prognosis in SSc is internal organ involvement, and improvements in monitoring internal organ involvement are needed. Most treatments for SSc target specific symptoms and/or internal organ complications, and show only modest efficacy at improving patient outcome. SSc patients are classified into one of two groups, diffuse (dSSc) or limited (lSSc). Further sub-dividing patients into groups beyond those currently recognized may direct drug treatments and improve determination of prognosis. A focus of the research presented here was to determine through genome-wide gene expression analysis of dSSc skin if subsets of patients were consistently detectible and how these subsets would change over time. ...
This study analysed the mortality of SSc patients in the prospective multinational EUSTAR cohort. The dataset comprised the MEDS data and a structured questionnaire.7 The results demonstrate a high prevalence of disease-related causes of death. The prevalence of SSc-related causes of mortality is similar, or in between the figures of other cohorts.3 5 Our findings underscore the high prevalence of pulmonary (interstitial lung disease and PAH), as well as myocardial causes.9 10. Since the introduction of ACE inhibitors, renal crisis appears to have become an increasingly less frequent terminal event.5 In our cohort, renal crisis accounted for 4% of the deaths, similar to the recent data from Pittsburgh.5 Except one individual, all patients dying from renal crisis were on an ACE inhibitor at the time of death (data not shown). Prednisone equivalents above 15 mg daily has been implicated in exacerbating scleroderma renal crisis, but in our study only one patient was on such treatment in the 3 ...
Do you have systemic sclerosis-associated pulmonary arterial hypertension (PAH)? Our researchers are trying to determine if a new medication, Tecfidera®, helps improve quality of life for patients with this disease. Learn more.
Hemodialysis and renal transplantation were done in a patient with progressive systemic sclerosis and renal failure. The patients clinical course following transplantation was uncomplicated, and a biopsy of the renal allograft done 14 months after transplantation did not show recurrence of the original disease. These observations favor the consideration of early renal transplantation in patients with scleroderma and renal failure. ...
BACKGROUND. Scleroderma is an autoimmune disease with a characteristic vascular pathology. The vasculopathy associated with scleroderma is one of the major contributors to the clinical manifestations of the disease. METHODOLOGY/PRINCIPAL FINDINGS. We used immunohistochemical and mRNA in situ hybridization techniques to characterize this vasculopathy and showed with morphometry that scleroderma has true capillary rarefaction. We compared skin biopsies from 23 scleroderma patients and 24 normal controls and 7 scleroderma patients who had undergone high dose immunosuppressive therapy followed by autologous hematopoietic cell transplant. Along with the loss of capillaries there was a dramatic change in endothelial phenotype in the residual vessels. The molecules defining this phenotype are: vascular endothelial cadherin, a supposedly universal endothelial marker required for tube formation (lost in the scleroderma tissue), antiangiogenic interferon α (overexpressed in the scleroderma dermis) and ...
Table 1: Improvement of Mouth Functional Disability in Systemic Sclerosis Patients over One Year in a Trial of Fat Transplantation versus Adipose-Derived Stromal Cells
Loop recorder may detect cardiac conduction abnormalities in systemic sclerosis patients in European League Against Rheumatism (EULAR) 2016 Annual Congress | MIMS Malaysia
Scleroderma (systemic sclerosis) is associated with several autoantibodies, each of which is useful in the diagnosis of affected patients and in determining their prognosis. Anti-centromere antibodies (ACA) and anti-Scl-70 antibodies are very useful in distinguishing patients with systemic sclerosis (SSc) from healthy controls, from patients with other connective tissue disease, and from unaffected family members. Whereas ACA often predict a limited skin involvement and the absence of pulmonary involvement, the presence of anti-Scl-70 antibodies increases the risk for diffuse skin involvement and scleroderma lung disease. Anti-fibrillarin autoantibodies (which share significant serologic overlap with anti-U3-ribonucleoprotein antibodies) and anti-RNA-polymerase autoantibodies occur less frequently and are also predictive of diffuse skin involvement and systemic disease. Anti-Th/To and PM-Scl, in contrast, are associated with limited skin disease, but anti-Th/To might be a marker for the development of
PubMed journal article: Low occurrence of digital ulcers in scleroderma patients treated with bosentan for pulmonary arterial hypertension: a retrospective case-control study. Download Prime PubMed App to iPhone, iPad, or Android
What is scleroderma? Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body. This inflammation leads to patches of tight, hard skin. Scleroderma involves many systems in your body. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage. There are two major types of scleroderma: Localized scleroderma only affects the skin and the structures directly under the skin. Systemic scleroderma, also called systemic sclerosis, affects many systems in the body. This is the more serious type of scleroderma and can damage your ...
What is scleroderma? Scleroderma is an autoimmune connective tissue and rheumatic disease that causes inflammation in the skin and other areas of the body. This inflammation leads to patches of tight, hard skin. Scleroderma involves many systems in your body. A connective tissue disease is one that affects tissues such as skin, tendons, and cartilage. There are two major types of scleroderma: Localized scleroderma only affects the skin and the structures directly under the skin. Systemic scleroderma, also called systemic sclerosis, affects many systems in the body. This is the more serious type of scleroderma and can damage your ...
TY - JOUR. T1 - Inter and intraobserver variability of total skin thickness score (Modified Rodnan TSS) in systemic sclerosis. AU - Clements, P.. AU - Lachenbruch, P.. AU - Siebold, J.. AU - White, B.. AU - Weiner, S.. AU - Martin, R.. AU - Weinstein, A.. AU - Weisman, M.. AU - Mayes, M.. AU - Collier, D.. AU - Wigley, F.. AU - Medsger, T.. AU - Steen, V.. AU - Moreland, L.. AU - Dixon, M.. AU - Massa, M.. AU - Lally, E.. AU - McCloskey, D.. AU - Varga, J.. PY - 1995. Y1 - 1995. N2 - Objective. Assessment of the inter and intraobserver variability of the modified Rodnan (m-Rodnan) total skin thickness score by clinical palpation [a commonly used outcome measure in trials of systemic sclerosis (SSc)]. Methods. Skin thickness was assessed by clinical palpation of 17 body areas on a 0 to 3 scale (normal, mild, moderate, severe). The m-Rodnan total skin thickness score was derived by summation of the scores from all 17 body areas. Using the m-Rodnan, 6-7 investigators assessed skin thickness in 5-6 ...
Diffuse scleroderma is a type of scleroderma characterized as a systemic (internal) chronic connective tissue disease that is visible through the hardening of the skin (please also see: Scleroderma). Scleroderma can be diffuse or limited. In diffuse scleroderma, skin thickening and hardening occurs more rapidly and involves more skin areas than in limited disease. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a persons mouth and increases the importance of good dental care. Skin may gain or lose pigment (color) creating light and dark patches. In addition, people with diffuse scleroderma have a higher risk of developing sclerosis or fibrous hardening of the internal organs such as the heart, lungs and kidneys. The amount of organ involvement is highly variable; some individuals have ...
TY - CHAP. T1 - Systemic Sclerosis (Scleroderma) and Raynauds Phenomenon. AU - Pope, Janet E.. AU - Clements, Philip J.. AU - Furst, Daniel E.. AU - Hummers, Laura K.. AU - Khanna, Dinesh. AU - Mayes, Maureen D.. AU - Medsger, Thomas. AU - Seibold, James. AU - Steen, Virginia. PY - 2009/12/1. Y1 - 2009/12/1. N2 - Systemic sclerosis (scleroderma; SSc) is a chronic con nective tissue disease characterized by inflammation within, and fibrosis of, the skin, vascular abnormalities, visceral damage, and the production of autoantibodies. SSc is divided generally into limited and diffuse forms, based on the extent of skin involvement. A group of conditions known as localized sclero-derma, which includes morphea, linear scleroderma, and en coup de sabre, is discussed in the chapter on Scleroderma Mimickers (Chap. 11). Diffuse SSc has a greater extent of skin involvement and is more likely to be associated with renal crisis, pulmonary fibrosis, and cardiomyopathy. Patients with diffuse SSc have a ...
Sideline Spouses Being my wifes biggest fan doesnt seem like enough. My wife has Scleroderma. Never heard of it? Thats OK, spell check hasnt either. So what is it? Scleroderma is an autoimmune disease that involves the hardening and tightening of the skin and connective tissues. There are two main branches of Scleroderma, neatly packaged for you in this chart that can also be found on the Scleroderma Foundation website.. In the short of it, localized Scleroderma generally affects areas of the skin and muscle while systemic scleroderma not only affects areas of the skins and muscle but also internal organs and the esophagus and can lead to sclerosis, or hardening, of the internal organs.. My wife has localized scleroderma and of the two major branches of localized she has both morphea and linear, as well as en coup de sabre which forms a crease or line on her face. She was diagnosed when she was six years old and was the first child officially recognized with the disease. She became the ...
TY - JOUR. T1 - T lymphocyte abnormalities in juvenile systemic sclerosis patients. AU - Reiff, Andreas. AU - Weinberg, Kenneth I.. AU - Triche, Timothy. AU - Masinsin, Bernadette. AU - Mahadeo, Kris M.. AU - Lin, Chuan Hao. AU - Brown, Diane. AU - Parkman, Robertson. N1 - Copyright: Copyright 2013 Elsevier B.V., All rights reserved.. PY - 2013/10. Y1 - 2013/10. N2 - Multi-center evaluations of pediatric patients with juvenile systemic sclerosis (jSSc) have suggested that the pathogenesis of jSSc may differ from that of systemic sclerosis (SSc) in adult patients. Therefore, we undertook to identify abnormalities in the T lymphocytes of jSSc patients and to determine if they differed from the abnormalities reported in the T lymphocytes of adult SSc patients. We identified decreases in the frequency of resting regulatory T lymphocytes and an increased frequency of CD45RA expressing effector memory (EMRA) CD4 T lymphocytes, which were characterized by an increased frequency of CCR7 protein ...
Clinical Features of Idiopathic Interstitial Pneumonia with Systemic Sclerosis-Related Autoantibody in Comparison with Interstitial Pneumonia with Systemic Sclerosis. . Biblioteca virtual para leer y descargar libros, documentos, trabajos y tesis universitarias en PDF. Material universiario, documentación y tareas realizadas por universitarios en nuestra biblioteca. Para descargar gratis y para leer online.
Anticentromere antibodies have been associated with peripheral vascular occlusive disease, most frequently accompanied by sclerodactyly in the context of a connective tissue disorder. We report a case of digital gangrene with no other clinical associations except positive anticentromere antibodies. Our patient, a 53-year-old Caucasian woman, non-smoker, presented with progressive pain and blackening of the distal right third finger over the preceding five weeks. No sclerodactyly was evident. She was anticentromere antibody positive at greater than 100 U/mL. Angiography revealed diffuse distal vasculopathy in both upper extremities. Other investigations were unremarkable. It is rare for anticentromere antibody-associated digital necrosis to develop without concomitant sclerodactyly. However, this patients case illustrates the need to consider an autoimmune contribution to the pathogenesis of digital ischemia even in the absence of a recognizable connective tissue disease.
A case is reported of progressive systemic sclerosis with pulmonary fibrosis which was complicated by recurrent haemoptyses due to diffuse pulmonary haemorrhage. We have found no other report of this association. The haemorrhage finally remitted after treatment was started with 40 mg prednisone daily, though previously spontaneous remissions had occurred.. ...
TY - JOUR. T1 - The analysis of antinuclear and antinucleolar autoantibodies of scleroderma by radioimmunoprecipitation assays. AU - Kipnis, Robert J.. AU - Craft, Joe. AU - Hardin, John A.. PY - 1990/9. Y1 - 1990/9. N2 - We have characterized autoantibodies to nuclear and nucleolar antigens in 112 patients with diffuse scleroderma, CREST syndrome (calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias), scleroderma overlap syndromes, or primary Raynauds phenomenon by indirect immunofluo‐rescence and radiolabeled immunoprecipitation assays. We noted for the first time that anti‐Th RNP antibodies represent a common antibody specificity in scleroderma (occurring in 13% of patients with scleroderma‐like illnesses) and that anti‐NOR 90 antibodies are quite rare in American patients (none found). In addition, we describe 3 new scleroderma‐associated autoantibodies.. AB - We have characterized autoantibodies to nuclear and nucleolar antigens in 112 patients ...
Methods Fifty consecutive SSc patients recruited at two Rheumatology Units since January 2003, who received iloprost infusions for a time period ,2 years were included in the analysis. Patients features were: males/females ratio 7/43; mean age at SSc diagnosis 43.5±12.7SD years; median disease duration at the beginning of therapy 2 years (range 0-18); mean follow-up with iloprost treatment 10±4.2SD years; diffuse SSc cutaneous subset in 13/50 (30%) patients; anti-Scl70 and anticentromere autoantibodies in 25 (50%), and 14 (28%) subjects, respectively. For all patients epidemiological, clinical, laboratory, and instrumental data were available for the study period. Iloprost schedule consisted in monthly i.v. infusion at 0.8-1 ng/kg body weight/min with average cumulative dosage per each session of 25 μg, according to patients tolerance. At the beginning of the treatment and in the presence of recent onset, severe and/or multiple DU at high risk of gangrene, patients were hospitalized for ...
Figure 3: Skin biopsy from the face showing dermal shrinkage.. Discussion. Progressive systemic sclerosis is an autoimmune disease of unknown etiology with an estimated annual incidence of 19 new cases per million adults per year.6 It is characterized by three major processes: disease specific autoantibodies, organ fibrosis, and small vessel vasculopathy. There is a female preponderance towards the 30-50 years age group.7 Patients can be classified into two principal subsets defined largely by the pattern of skin involvement, as well as clinical and laboratory manifestations. Diffuse cutaneous SSc is associated with progressive skin induration, starting in the fingers and ascending from the distal to proximal extremities, the face, and the trunk. These patients are at risk of early pulmonary fibrosis and acute renal involvement. Patients with limited cutaneous SSc (lcSSc) generally have long-standing Raynauds phenomenon before other manifestations of SSc appear. Skin involvement in lcSSc is ...
CREST syndrome, also known as the limited cutaneous form of systemic sclerosis (lcSSc) is a multisystem connective tissue disorder. The acronym CREST refers to the five main features: calcinosis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. It is associated with detectable antibodies against centromeres (a component of the cell nucleus), and usually spares the kidneys (a feature more common in the related condition systemic scleroderma). If the lungs are involved, it is usually in the form of pulmonary arterial hypertension. CREST causes thickening and tightening of the skin with deposition of calcific nodules (calcinosis). Raynauds phenomenon is frequently the first manifestation of CREST/lcSSc, preceding other symptoms by years. Stress and cold temperature induce an exaggerated vasoconstriction of the small arteries, arterioles, and thermoregulatory vessels of the skin of the digits. Clinically this manifests as a white-blue-red transitions in skin ...
Background/Purpose: Interstitial lung disease (ILD) is a common complication of systemic sclerosis (SSc) and is a leading contributor to mortality in SSc patients. Once lung fibrosis occurs, lung function disease course may become stable or progressively decline. While some demographic and SSc-related factors have been associated with development of ILD, little is known about what contributes to progression. We studied the clinical manifestations of SSc-gastroesophageal (GE) involvement in relation to ILD status to determine associations between GE involvement and ILD progression in SSc. Our objective was to determine if GE reflux and dysphagia are associated with progressive ILD as measured by PFTs over three years. Methods: Canadian Scleroderma Research Group (CSRG), a multi-center database of adult SSc patients, annually evaluates and collects patient information including demographics, skin manifestations, internal organ involvement and function assessment data. Using indicators of GE ...
Background: Systemic sclerosis is a chronic disease of connective tissue accompanied with increased risk of foot ulcers. Biomechanical indexes (soft tissue thickness and compressibility) could affect the risk of this phenomenon.Objective: The aim of this study was assessment of heel pad and first metatarsal head soft tissue thickness and compressibility index in scleroderma patients with and without foot ulcers and comparison with healthy individuals.Methods: Heel pad thickness in standing(loaded) and lying(unloaded) positions was measured in 40 scleroderma patients by means of lateral foot radiography. Compressibility index was measured as the ratio of loaded to unloaded thickness. Also, soft tissue thickness of first metatarsal head was measured with ultrasound. Results were compared with 40 healthy controls of matched age and body mass index.Results: Among 40 scleroderma patients (36 females, 4 males) with mean age of 45 (±12) years and mean body mass index of 25.5 (±4) and mean disease duration of
Introduction: Scleroderma is an autoimmune disease characterized by inflammation, vasculopathy, and fibrosis of skin, vasculative and internal organs. Delayed wound healing is a known complication of scleroderma. The purpose of this study was to investigate whether chronic wounds in scleroderma patients heal more slowly than chronic wounds of other etiology. Methods: This research was conducted through the Wound Etiology and Healing Study (WE-HEAL Study). The WE-HEAL Study is a biospecimen and data repository approved by the George Washington University IRB (041408). Subjects gave written informed consent for collection of their data. Scleroderma cases with wounds (n=25) and age and sex matched control patients with chronic wounds from other etiologies (n=25) were selected for analysis. Baseline demographics, comorbidities, wound size, time to healing, and pain score were compared between the two groups. Scleroderma wounds were further analyzed based on scleroderma classification (localized vs. systemic
Ive not been given any specific treatments for this auto-immune condition that crept into my body maybe as many as twenty years ago; diffuse systemic sclerosis. There, its typed out in hard-edged letters. It was formally diagnosed back in 2001. I had a brief look on the internet, but it filled me with such dismay that I soon stopped doing that. Instead, I decided to focus on my life as it is NOW, rather than worrying about a life that might never come to pass. I trundled to the specialists every year, had regular tests, and was told that the disease is progressing so slowly that no treatment would be necessary ...
INTRODUCTION In patients with systemic sclerosis (SSc), associated pulmonary arterial hypertension (SSc-APAH) is the leading cause of death. The objective of this prospective screening study was to analyse sensitivity and specificity of stress Doppler echocardiography (SDE) in detecting pulmonary hypertension (PH). METHODS Pulmonary artery pressures and further parameters of PH were assessed by echocardiography and right heart catheterisation (RHC) at rest and during exercise in patients with SSc. Investigators of RHC were blinded to the results of non-invasive measurements. RESULTS Of 76 patients with SSc (64 were female and mean age was 58±14 years), 22 (29 %) had manifest PH confirmed by RHC: four had concomitant left heart diseases, three had lung diseases, and 15 had SSc-APAH. Echocardiography at rest missed PH diagnosis in five of 22 patients with PH when a cutoff value for systolic pulmonary arterial pressure (PASP) was more than 40 mm Hg at rest. The sensitivity of echocardiography at rest
Having a disease that nobody has heard of is a lonely business. When even the doctors cannot recognise it, or tell you what is going to happen, it is lonelier still. So Scleroderma Awareness Day was created to tell people, including the medical community, what it means to have this disabling disease. June 29 is a day to recognise the bravery of those who live with scleroderma, and to demand equal treatment and equal care for people with scleroderma across Europe.. In February 2010 the1ST SYSTEMIC SCLEROSIS WORLD CONGRESS was held in Florence, Italy, with participants from countries from all over the world. At the world congress it was agreed that June 29th would be celebrated all over the world. Scleroderma day grew from zero to world scleroderma day in 2 years, and is celebrated in countries in Europe, Australia, Canada, Brazil, India and many more countries.. ...
Objective. To determine serum levels of tumor necrosis factor-related weak inducer of apoptosis (TWEAK) and its clinical associations in patients with systemic sclerosis (SSc).. Methods. Serum TWEAK levels from 70 patients with SSc were examined by ELISA. In a retrospective longitudinal study, sera from 23 patients with SSc were analyzed (followup 0.8-7.2 yrs).. Results. Serum TWEAK levels were elevated in patients with SSc (n = 70) compared with healthy controls (n = 31) and patients with systemic lupus erythematosus (n = 22). Among patients with SSc, there were no differences in serum TWEAK levels between limited cutaneous SSc and diffuse cutaneous SSc. Patients with SSc who had elevated TWEAK levels less often had pulmonary fibrosis and decreased vital capacity than those with normal TWEAK levels. In the longitudinal study, SSc patients with inactive pulmonary fibrosis or without pulmonary fibrosis consistently exhibited increased TWEAK levels, while those with active pulmonary fibrosis ...
TY - JOUR. T1 - Evidence for chronic inflammation as a component of the interstitial lung disease associated with progressive systemic sclerosis. AU - Rossi, G. A.. AU - Bitterman, P. B.. AU - Rennard, S. I.. AU - Ferrans, V. J.. AU - Crystal, R. G.. PY - 1985. Y1 - 1985. N2 - Progressive systemic sclerosis (PSS) is a generalized disorder characterized by fibrosis of many organs including the lung parenchyma. Unlike most other interstitial disorders, traditional concepts of the interstitial lung disease associated with PSS have held it to be a pure fibrotic disorder without a significant inflammatory component. To directly evaluate whether an active alveolitis is associated with this disorder, patients with chronic interstitial lung disease and PSS were studied by open lung biopsy, gallium-67 scanning, and bronchoalveolar lavage. Histologic evaluation of the biopsies demonstrated that the interstitial fibrosis of PSS is clearly associated with the presence of macrophages, lymphocytes, and ...
Dr. Shane Shapera, discusses the treatment of Scleroderma interstitial lung disease (SSc-ILD). At the end of this talk, listeners will be able to describe what Scleroderma interstitial lung disease means and better understand the risk factors for SSc-ILD development and its progression and the evidence for and against anti-inflammatory and anti-fibrotic treatment of SSc-ILD.
Diverse internal and external pathologic stimuli can trigger cellular stress response pathways (CSRPs) that are usually counteracted by intrinsic homeostatic machinery, which responds to stress by initiating complex signaling mechanisms to eliminate either the stressor or the damaged cells. There is growing evidence that CSRPs can have context-dependent homeostatic or pathologic functions that may result in tissue fibrosis under persistence of stress. CSRPs can drive intercellular communications through exosomes (trafficking and secretory pathway determinants) secreted in response to stress-induced proteostasis rebalancing. The injured tissue environment upon sensing the stress turns on a precisely orchestrated network of immune responses by regulating cytokine-chemokine production, recruitment of immune cells, and modulating fibrogenic niche and extracellular matrix (ECM) cross-talk during fibrotic pathologies like cardiac fibrosis, liver fibrosis, laryngotracheal stenosis, systemic scleroderma,
The Research Grant Program is vital to help forward research on scleroderma, said Carol Feghali-Bostwick, Ph.D., researcher and Vice Chair of the Foundations Board of Directors and head of the research committee. We also hope that the program helps motivate new researchers to become interested in investigating scleroderma.. The two highest scoring research proposals received the Foundations Marta Marx and Mark Flapan awards. The Marta Marx Fund Eradication of Scleroderma Award went to Dr. Wu from the University of Miami. The award is funded by bequests from Marta Marx, who had scleroderma, and her brother Rudolph Juhl. The Mark Flapan Award was awarded to Dr. Koch from the University of Michigan. It is named in honor of the late psychologist and scleroderma patient.. The Walter A. Coyle Memorial Research Grant Award, which is made possible through the generosity of the New England Chapter, was presented to Dr. Trojanowska of Boston University School of Medicine. This is the third research ...
I have been asked to share my story many times and I always struggle to start writing. I am a writer, researcher and communicator yet I struggle to tell my story, why? My block stems from my belief that I am not defined by my body or by my job.. I am a 41 year old woman living with a rare chronic illness called Essential Mixed Cryoglobulinemia (EMC). I have been formally diagnosed with EMC since I was 26 years old but I believe that I have had this disease since I was a young child.. I had 6 broken ankles between fourth and eight grades; all of them happened during the winter months when I was exercising, walking or using stairs. But in sharp contrast to my athletic days there were days I could do nothing because I was in so much pain or so tired. I lay in bed and slept to recover. My mama supported me with unquestioning faith. When I hurt or was tired I told her and she believed me.. After graduating college I was misdiagnosed with Progressive Systemic Scleroderma. I began to take ...
Abdominal Cramps, Constipation, Oral Mucosal Disorder Symptom Checker: Possible causes include Systemic Scleroderma, Anorexia Nervosa, Malnutrition. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.
Morphology. Virtually all organs are involved.. 1. Skin. Bundles of the collagen bind the skin to the underlying tissue.. Changes take place in three stages.. Stage I: There is edema which is non-pitting and microscopically there is edema, perivascular lymphocytic infiltrate, capillaries and small arteries are thickened, and there is collagen deposition.. Stage II: Due to an increase in collagen formation, wrinkles disappear. The epidermis becomes thin.. Stage III: This is the atrophic stage where claw-like hand, the face is drawn and mask. Microscopically there is atrophy of the epidermis and increase collagen. Calcification may be seen.. In the late stages due to loss of blood supply ulceration may take place.. Skin involvement may be a part of CREST syndrome. Where:. C = Calcinosis.. R = Raynauds phenomenon.. E = Esophageal involvement.. S = sclerodactyly (localized sclerosis of the finger).. T = Telangiectasia.. 2. Gastrointestinal. Gastrointestinal involvement is seen ,50% of the cases. ...
Scleroderma also known as systemic sclerosis, is a systemic autoimmune disease characterized by damage to endothelial and smooth muscle cells of the small arteries. Replacement with fibrous material results, and there is an influx of inflammatory cells. Primarily affects skin and joints but can affect the heart and lungs, and digestive tract. Some types of scleroderma are rapidly progressive and deadly. Scleroderma is often treated with immunosuppressive drugs, and there is no known cure. Some investigators are looking at using the regenerative properties of cell therapy to mitigate the impact of scleroderma. Research is ongoing to evaluate the effects of stem cells on auto-immune conditions. As recently stated in Best Practices & Research Clinical Rheumatology in a review of Scleroderma, Stem cell transplantation seems to be promising in restarting the immune system to diminish fibrosis and restore microvasculature. Read More…. ...
The Scleroderma Clinical Trials Consortium Research Roundtable. The Scleroderma Clinical Trials Consortium (SCTC) is an international consortium representing the vast majority of researchers and clinicians who have particular interest and expertise in the care of, and research in, scleroderma (systemic sclerosis). The goal of the SCTC is to conduct, sponsor, or facilitate clinical research projects that lead to advances in the management, treatment, and outcomes of patients with scleroderma. The SCTC seeks to ensure that research in scleroderma adheres to high standards for design, conduct, and reporting of results. The SCTC is particularly focused on improving and developing outcome measures for clinical trials and observational studies, participating in the design and conduct of clinical trials and observational studies, and helping to improve the efficiency of clinical trials. The SCTC strongly encourages international collaboration, inclusiveness in research groups, and involvement of ...
In 1997 I was diagnosed with scleroderma and given a 15month prognosis. In 1998 I became a patient at the Scleroderma Unit. The cause and cure to both Scleroderma and Raynauds remain unknown. Please donate to help fund medical research where 100% of your donation will be used for research purposes only. The Royal Free Hospital has the largest centre specialising in scleroderma in the UK led by Professor Chris Denton. #SclerodermaFreeWorld #RaynaudsFreeWorld #Research. ...
9th ed. Philadelphia, Pa: Saunders Elsevier; 2012: Chap 84.. Burt RK, Shah SJ, Dill K, et al. Autologous non-myeloablativehaemopoietic stem-cell transplantation compared with pulse cyclophosphamide once per month for systemic sclerosis (ASSIST): an open-label, randomised phase 2 trial. Lancet. 2011;6;378(9790):498-506.. Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49(2):271-280.. Elhai M, Meune C, Avouac J, et al. Trends in mortality in patients with systemic sclerosis over 40 years: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) [serial online]. September 2011.. Henness S, Wigley FM. Current drug therapy for scleroderma and secondary Raynauds phenomenon: evidence-based review. Curr Opin Rheumatol. 2007;19:611-618.. Kowal-Bielecka O, Landewe R, Avouac J. EULAR Recommendations for the treatment of systemic sclerosis: a report from the EULAR ...
In 1997 I was diagnosed with scleroderma and given a 15month prognosis. In 1998 I became a patient at the Scleroderma Unit. The cause and cure to both Scleroderma and Raynauds remain unknown. Please donate to help fund medical research where 100% of your donation will be used for research purposes only. The Royal Free Hospital has the largest centre specialising in scleroderma in the UK led by Professor Chris Denton. #SclerodermaFreeWorld #RaynaudsFreeWorld #Research. ...
Scleroderma, also known as systemic sclerosis, is a chronic systemic autoimmune disease characterised by hardening (sclero) of ... "Scleroderma". Medscape Reference. WebMD. Retrieved 5 March 2014. Hajj-ali RA (June 2013). "Systemic Sclerosis". Merck Manual ... and blockade of the mTORC pathway is under investigation as a treatment for scleroderma. mTOR inhibitors, e.g. rapamycin, are ... Implications for Scleroderma". The Journal of Investigative Dermatology. 135 (11): 2873-6. doi:10.1038/jid.2015.252. PMC ...
It can occur alone or with rheumatoid arthritis, scleroderma, or systemic lupus erythematosus. Nine out of 10 cases occur in ... "Systemic scleroderma". Genetics Home Reference. Retrieved 2019-11-19. Reference, Genetics Home. "Sjögren syndrome". Genetics ... such as systemic lupus erythematosus or scleroderma. Latent lupus and incomplete lupus are alternative terms that have been ... such as systemic lupus erythematosus (SLE); systemic sclerosis (SSc); dermatomyositis (DM); polymyositis (PM); anti-synthetase ...
Majeed M, Al-Mayouf SM, Al-Sabban E, Bahabri S (2000). "Coexistent linear scleroderma and juvenile systemic lupus erythematosus ... Frontal linear scleroderma (also known as en coup de sabre or morphea en coup de sabre) is a type of linear scleroderma ... Linear scleroderma is a type of localised scleroderma which is an autoimmune disease characterized by a line of thickened skin ... Morphea is a form of scleroderma that is more common in women than men, in a ratio 3:1. Morphea occurs in childhood as well as ...
It can also be a feature of systemic scleroderma. Hagberg and Benumof's Airway Management (4e ed.). Elsevier. pp. 608-639. ...
Journal of Scleroderma and Related Disorders. 3 (2): 132-152. doi:10.1177/2397198318758606. One Day, Two Dollars FDA > CDRH > ... "Therapeutic plasma exchange for the treatment of systemic sclerosis: A comprehensive review and analysis". ... scleroderma) Thrombotic thrombocytopenic purpura (TTP) / hemolytic uremic syndrome Toxic epidermal necrolysis (TEN) Transverse ... vulgaris Recurrent focal and segmental glomerulosclerosis in the transplanted kidney Refsum disease Rhabdomyolysis Systemic ...
This systemic disease resembles scleromyxedema and to some extent scleroderma. It may occur months after contrast has been ... Marckmann, P.; Skov, L.; Rossen, K.; Dupont, A.; Damholt, M.B.; Heaf, J.G.; Thomsen, H.S. (2006). "Nephrogenic Systemic ... a specific trigger for the development of nephrogenic fibrosing dermopathy and nephrogenic systemic fibrosis?". Nephrology ... a causal relation between the administration of gadolinium based contrast media and the development of nephrogenic systemic ...
The presentation is similar to that of scleroderma or systemic sclerosis. However, unlike scleroderma, eosinophilic fasciitis ... the characteristic and severe effects of scleroderma and systemic sclerosis, such as Raynaud's syndrome, involvement of the ... Falanga, Vincent; Medsger, Thomas A. (1987). "Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, ... localized scleroderma, and eosinophilic fasciitis". Journal of the American Academy of Dermatology. 17 (4): 648-656. doi: ...
Steen, V. D.; Medsger Jr, T. A.; Rodnan, G. P. (1982). "D-Penicillamine therapy in progressive systemic sclerosis (scleroderma ... Penicillamine has been used to treat scleroderma. Penicillamine can be used as a disease-modifying antirheumatic drug (DMARD) ... ISBN 978-1-4160-2973-1. Chalmers, A.; Thompson, D.; Stein, H. E.; Reid, G.; Patterson, A. C. (1982). "Systemic lupus ... Underwood, J. C. E. (2009). General and Systemic Pathology. Elsevier Limited. ISBN 978-0-443-06889-8. Taylor; Cumming; ...
... scleroderma): specific autoantigen genes are selectively overexpressed in scleroderma fibroblasts". Journal of Immunology. 167 ... Zhou X, Tan FK, Xiong M, Milewicz DM, Feghali CA, Fritzler MJ, Reveille JD, Arnett FC (Dec 2001). "Systemic sclerosis ( ... Antisera from approximately 8% of humans with the autoimmune disease scleroderma recognize fibrillarin. Fibrillarin is a ... in patients with systemic sclerosis". Arthritis and Rheumatism. 35 (1): 95-100. doi:10.1002/art.1780350114. PMID 1731817. ...
1999: possible role of parvovirus B19 and CMV in systemic sclerosis (scleroderma). 2. Serological studies and therapeutic ... and systemic sclerosis (scleroderma). In 1991, soon after the discovery of the hepatitis C virus (HCV), he demonstrated the ... Ferri C, et al: Parvovirus B19 infection of bone marrow in systemic sclerosis patients. Clin Exp Rheumatol 1999; 17(6):718-20. ... Since 1972, the majority of Ferri's work has been in the fields of systemic sclerosis, cryoglobulinemic vasculitis and other ...
In systemic lupus erythematosus ATA are associated with nephritis. Increases in ATA+ in scleroderma and SLE are associated with ... HLA-DR2 (DR15 and DR16) are associated with Scleroderma and systemic sclerosis. It has been found that patients with ATA that ... is a type of anti-nuclear autoantibody seen mainly in diffuse systemic scleroderma, but is also seen the more limited form of ... Scleroderma results from the overproduction of collagen in affected tissues, one study claims that there is an increased ...
"Type III collagen aminopropeptide levels in serum of patients with progressive systemic scleroderma". The Journal of ... Increased amounts of type III collagen are found in many fibrotic conditions such as liver and kidney fibrosis, and systemic ... The fourth mouse model with defective Col3a1 gene is the tight skin mouse (Tsk2/+), which resembles the human systemic ... "Co-ordinate increase in the expression of type I and type III collagen genes in progressive systemic sclerosis fibroblasts". ...
Luderschmidt C, Scholz S, Mehlhaff E, König G, Albert E (1987). "Association of progressive systemic scleroderma to several HLA ... systemic sclerosis, childhood epilepsy, early-onset alopecia areata, short-ragweed Ra6 allergy, primary antiphospholipid ... "Autoantibodies and immunogenetics in 30 patients with systemic sclerosis and their families". J Rheumatol. 14 (4): 760-5. PMID ...
There is some evidence implicating Mycoplasma hyorhinis in the pathogenesis of Scleroderma, a chronic systemic disease of ... One study found it has been isolated from Scleroderma patients. Another study found that injecting Mycoplasma hyorhinis into ... However, more research is needed to confirm or refute the link between Mycoplasma hyorhinis and Scleroderma. Antibiotics are ... "Mycoplasma infection induces a scleroderma-like centrosome autoantibody response in mice". Clin Exp Immunol. 137 (2): 288-97. ...
Redness and scleroderma-like induration occurs on the skin. In addition, the mobility of the lips, hands, arms, and legs is ... and hoarseness are among the notable systemic symptoms. If viscera is involved, the disease will be fatal. The dermatoneuro ...
The systemic disease nephrogenic systemic fibrosis (NSF) resembles scleromyxedema and to some extent scleroderma. It may occur ...
... of patients with limited systemic scleroderma and in 15% of those with the diffuse form of scleroderma. The specificity of this ... They occur in some autoimmune diseases, frequently in limited systemic scleroderma (formerly called CREST syndrome), and ... a positive anti-centromere antibody finding is strongly suggestive of limited systemic scleroderma. Anti-centromere antibodies ... occasionally in the diffuse form of scleroderma. They are rare in other rheumatic conditions and in healthy persons. Anti- ...
These disorders include: systemic lupus erythematosus (SLE), rheumatoid arthritis, Sjögren syndrome, scleroderma, polymyositis ... Barnett, AJ; McNeilage, LJ (May 1993). "Antinuclear antibodies in patients with scleroderma (systemic sclerosis) and in their ... Anti-Scl-70 antibodies are linked to scleroderma. The sensitivity of the antibodies for scleroderma is approximately 34%, but ... In addition to scleroderma, these antibodies are found in approximately 5% of individuals with SLE. The antigenic target of ...
Watermelon stomach also occurs particularly with scleroderma, and especially the subtype known as systemic sclerosis. A full ... Research in 2010 has shown that anti-RNA polymerase III antibodies may be used as a risk marker for GAVE in systemic sclerosis ... Scleroderma Association website Archived 2015-05-07 at the Wayback Machine. Accessed September 29, 2010. Marie, I.; Ducrotte, P ... Ingraham, KM; O'Brien, MS; Shenin, M; Derk, CT; Steen, VD (2010). "Gastric antral vascular ectasia in systemic sclerosis: ...
When Jackson vomits blood, the diagnosis reaches systemic scleroderma, which is very difficult to treat. However, Thirteen ...
... or a concomitant connect tissue disorder such as systemic lupus erythematosus, rheumatoid arthritis, or scleroderma. 2. ... There may also be a history of decrease in salivary volume secondary to a systemic disease. A unilateral or bilateral painful ... or scleroderma). Oral candidiasis may also be present. Common key diagnostic factors 1. Fever - may present with an acute ... scleroderma, or dermatomyositis. 5. Oral candidiasis - may be present in cases of Sjogren's syndrome or in associate with a ...
It also is related to a number of autoimmune diseases, prominently systemic lupus erythematosus (SLE). Also found with Sjögren ... syndrome, rheumatoid arthritis, inherited complement deficiencies (esp C2 deficiency), scleroderma, Celiac disease. The ...
In systemic scleroderma, the incidence has been estimated to be 8 to 12% of all patients; in rheumatoid arthritis it is rare. ... However, in systemic lupus erythematosus it is 4 to 14%, and in sickle cell disease, it ranges from 20 to 40%. Up to 4% of ... Signs of systemic congestion resulting from right-sided heart failure include jugular venous distension, ascites, and ... Mittoo, Shikha (2014). "Pulmonary Manifestations of Systemic Lupus Erythematosus". Seminars in Respiratory and Critical Care ...
Trichloroethylene can cause scleroderma which is a systemic autoimmune disease that causes joint pain, skin stiffness, and ...
"The HLA-DR and DQ genes control the autoimmune response to DNA topoisomerase I in systemic sclerosis (scleroderma)". J. Clin. ... DR52 serotype is positively associated with systemic sclerosis, inflammatory myopathies, inclusion body myositis, DRB3*01 is ... II alleles associations of anticardiolipin and antibeta2GPI antibodies in a large series of European patients with systemic ...
Systemic autoimmune diseases include coeliac disease, lupus erythematosus, Sjögren syndrome, sarcoidosis, scleroderma, ... In systemic lupus there are autoantibodies to DNA, which cannot evoke a T cell response, and limited evidence for T cell ... Autoimmune diseases can be broadly divided into systemic and organ-specific or localised autoimmune disorders, depending on the ... Agmon-Levin, Nancy; Theodor, Emanuel; Segal, Ramit Maoz; Shoenfeld, Yehuda (2013-10-01). "Vitamin D in Systemic and Organ- ...
ISBN 0-7216-2921-0. Takehara K, Sato S (2005). "Localized scleroderma is an autoimmune disorder". Rheumatology. 44 (3): 274-9. ... "Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis". Annals of Internal ... 173 polymorphism of the macrophage migration inhibitory factor gene in systemic-onset juvenile idiopathic arthritis". Arthritis ...
... without systemic disease can be a result of trauma or cold. Panniculitis with systemic disease can be caused by[ ... citation needed]: connective tissue disorders such as lupus erythematosus or scleroderma; lymphoproliferative disease such as ... Erythema nodosum is a form of panniculitis characterised by tender red nodules, 1-10 cm, associated with systemic symptoms ... Forms associated with use of high doses of systemic corticosteroids during rapid corticosteroid withdrawal, and from the ...
Onset of this disease can be attributed to steroid usage, systemic lupus erythematosus, or trauma. In some cases, it is ... or scleroderma. It can also afflict patients living with congenital short digits or atypical anatomical epiphyseal blood supply ... Onset of Dieterich's disease can possibly be attributed to steroid usage, trauma, systemic lupus erythematosus, renal ...
Saville is a systemic Scleroderma patient and is very active in raising funds to find a cure for this disease. He sits on the ... "Scleroderma Fundraiser at HOB". lasvegassun.com. Retrieved August 18, 2016. "Scleroderma Research Foundation's Fundraiser at ... "Strip Scribbles: Scleroderma event raises $300K; 'All By Myself' video; Hakkasan takes L.A". lasvegassun.com. June 11, 2016. ... Rose Hospital Foundation and Scleroderma Research Foundation. He was formerly one of the Founding board members of the Nevada ...
Dessinioti C, Katsambas A, Antoniou C (May-June 2014). "Hidradenitis suppurrativa (acne inversa) as a systemic disease". ... due to negligible systemic absorption.[47][150] Nadifloxacin and dapsone (category C) are other topical antibiotics that may be ... "Approaches to limit systemic antibiotic use in acne: Systemic alternatives, emerging topical therapies, dietary modification, ... It has shown no systemic absorption or associated antiandrogenic side effects.[134][135][136] In a direct head-to-head ...
Scleroderma. *Systemic scleroderma *Progressive systemic sclerosis. *CREST syndrome. *Overlap syndrome / Mixed connective ...
systemic lupus erythematosus. *tinea capitis. *loose anagen syndrome. *lichen planopilaris. *folliculitis decalvans ...
Systemic autoimmune diseases include SLE, Sjögren's syndrome, sarcoidosis, scleroderma, rheumatoid arthritis, cryoglobulinemic ... Systemic autoimmune disease. References[edit]. *^ Patt H, Bandgar T, Lila A, Shah N (2013). "Management issues with exogenous ... In systemic lupus there are autoantibodies to DNA, which cannot evoke a T cell response, and limited evidence for T cell ... HLA DR2 is strongly positively correlated with Systemic Lupus Erythematosus, narcolepsy[11] and multiple sclerosis, and ...
systemic lupus erythematosus. *tinea capitis. *loose anagen syndrome. *lichen planopilaris. *folliculitis decalvans ...
Asch S, Goldenberg G (March 2011). "Systemic treatment of cutaneous lichen planus: an update". Cutis. 87 (3): 129-34. PMID ... Narrow band UVB phototherapy or systemic PUVA therapy are known treatment modalities for generalized disease.[43] ... Lichenoid drug eruptions may be caused by a variety of systemic medications and share clinical features with oral LP. ... Eisen D (February 2002). "The clinical features, malignant potential, and systemic associations of oral lichen planus: a study ...
Potential systemic absorption of imiquimod, with negative effects on white blood cell counts overall, and specifically ...
... radiation can aggravate several skin conditions and diseases, including[71] systemic lupus erythematosus, Sjögren's ... and localized scleroderma.[44][45] In addition, UV light, in particular UVB radiation, has been shown to induce cell cycle ... or systemic treatments such as methotrexate and Soriatane.[104] ...
Systemic disease[edit]. Systemic disorders associated with aphthous-like ulceration[5] Behçet's disease. Celiac disease. Cyclic ... Aphthous stomatitis occurs in individuals with no associated systemic disease.[6] Persons with certain systemic diseases may be ... Systemic medications. Various, mostly modulating immune response. Prednisolone,[6] colchicine,[6] pentoxifylline,[6] ... A systemic cause is more likely in adults who suddenly develop recurrent oral ulceration with no prior history.[14] ...
Systemic disease[edit]. Systemic disorders associated with aphthous-like ulceration[5]. Behçet's disease. Celiac disease. ... Aphthous stomatitis occurs in individuals with no associated systemic disease.[6] Persons with certain systemic diseases may be ... A systemic cause is more likely in adults who suddenly develop recurrent oral ulceration with no prior history.[13] ... A systematic review found that no single systemic intervention was found to be effective.[6] Good oral hygiene is important to ...
... scleroderma, and drug sensitivities. Because of this, the LE cell test is now performed only rarely and is mostly of historical ... Wikimedia Commons has media related to Systemic lupus erythematosus.. Look up systemic lupus erythematosus in Wiktionary, the ... "Handout on Health: Systemic Lupus Erythematosus". The National Institute of Arthritis and Musculoskeletal and Skin Diseases. ... Systemic lupus erythematosus (SLE), also known simply as lupus, is an autoimmune disease in which the body's immune system ...
... in people with systemic scleroderma. Bosentan causes harm to fetuses and pregnant women must not take it, and women must not ... "Optimal management of digital ulcers in systemic sclerosis". Therapeutics and clinical risk management. 11: 939-47. doi:10.2147 ...
系统性硬皮病(英语:Systemic scleroderma) *进行性系统性硬皮病(英语:Progressive systemic
... and bullous systemic lupus erythematosus. ... Scleroderma/morphea. *Granuloma annulare. *Lichen sclerosis et ...
... scleroderma and type 1 diabetes are also seen in X-linked agammaglobulinemia (XLA). Recurrent bacterial and fungal infections ... "Immunodeficiency and autoimmunity: lessons from systemic lupus erythematosus". Trends Mol Med. 18 (2): 101-108. doi:10.1016/j. ...
systemic lupus erythematosus. *tinea capitis. *loose anagen syndrome. *lichen planopilaris. *folliculitis decalvans ...
systemic lupus erythematosus. *tinea capitis. *loose anagen syndrome. *lichen planopilaris. *folliculitis decalvans ...
In addition to the above-mentioned disorders, excessive deposition of collagen occurs in scleroderma. ... "Neutrophil function in systemic lupus erythematosus and other collagen diseases". Ann Rheum Dis. 41 (1): 33-38. doi:10.1136/ ...
people with certain serious connective tissue diseases such as scleroderma, which make them especially sensitive to the side ... as opposed to systemic therapies, such as chemotherapy, hormonal therapy, or immunotherapy. ...
Scleroderma/. Morphea. *Localized scleroderma *Localized morphea. *Morphea-lichen sclerosus et atrophicus overlap ... systemic lupus erythematosus. *tinea capitis. *loose anagen syndrome. *lichen planopilaris. *folliculitis decalvans ...
Scleroderma-like reaction to taxanes. References[edit]. *^ a b "Docetaxel Use During Pregnancy". Drugs.com. 4 June 2020. ... with an α half-life of mean 4.5 minutes is representative of distribution to peripheral compartments from the systemic ...
IP agonists are also to treat severe vasoconstriction in Raynaud's disease, Raynaud's disease-like syndromes, and scleroderma.[ ... "Hand Impairment in Systemic Sclerosis: Various Manifestations and Currently Available Treatment". Current Treatment Options in ...
subacute infective endocarditis, scleroderma, trichinosis, systemic lupus erythematosus (SLE), rheumatoid arthritis, psoriatic ... scleroderma, trichinosis, systemic lupus erythematosus (SLE), rheumatoid arthritis, psoriatic nails,[1] antiphospholipid ... They could be due to hitting the nail ("trauma"), a sign of inflammation in blood vessels all around the body ("systemic ...
... is sometimes associated with autoimmune and inflammatory diseases such as Hashimoto's thyroiditis, scleroderma, ... rheumatoid arthritis, type 1 diabetes mellitus, psoriasis, Addison's disease, pernicious anemia, alopecia areata, systemic ...
... progressive systemic sclerosis (PSS, also known as scleroderma), the pregnancy-associated HELLP (hemolysis, liver dysfunction, ... systemic disease such as systemic lupus erythematosus (SLE) or progressive systemic sclerosis (PSS) (6%), and malignancy (1%). ... These patients also remain at ongoing risk of non-kidney systemic complications of the disease. Despite its history of use in ... aHUS is not the only condition that causes systemic TMA, a fact that makes differential diagnosis essential. Historically, the ...
Scleroderma. *Systemic scleroderma *Progressive systemic sclerosis. *CREST syndrome. *Overlap syndrome / Mixed connective ...
Scleroderma. *Systemic scleroderma *Progressive systemic sclerosis. *CREST syndrome. *Overlap syndrome / Mixed connective ... July 1983). The Clinical Management of Systemic Lupus Erythematosus. New York: Grune & Stratton. p. 221. ISBN 978-0-8089-1543-0 ... Lahita, Robert G. (1987). Systemic Lupus Erythematosus. New York: John Wiley & Sons. p. 859. ISBN 978-0-471-87388-4. .. ... While these symptoms are similar to those of systemic lupus erythematosus, they are generally not as severe unless they are ...
... s have also been used in chronic kidney failure and kidney involvement in systemic sclerosis (hardening of tissues ... as scleroderma renal crisis). In those with stable coronary artery disease, but no heart failure, benefits are similar to other ...
Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. Explore symptoms, inheritance, ... In one type of systemic scleroderma, known as limited cutaneous systemic scleroderma, fibrosis usually affects only the hands, ... In another type of systemic scleroderma, known as diffuse cutaneous systemic scleroderma, the fibrosis affects large areas of ... In the third type of systemic scleroderma, called systemic sclerosis sine scleroderma ("sine" means without in Latin), fibrosis ...
Systemic scleroderma in children is very rare and is considered similar to adult-onset disease. In adults, new etiopathogenetic ... A controlled trial showing that interferon-alpha is of no value in the treatment of systemic scleroderma.PubMedCrossRefGoogle ... Results from this mail survey suggest a better outcome for juvenile systemic scleroderma.PubMedCrossRefGoogle Scholar ... An excellent review on stem cell transplantation in autoimmune diseases including systemic scleroderma.PubMedCrossRefGoogle ...
Systemic sclerosis (scleroderma) and pregnancy By Bonnie L Bermas, MD. Retrieved on Dec 13, 2009 "Juvenile Scleroderma Network ... Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and ... Masi AT (1980). "Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma ... and is thought to keep the disease at bay by reducing the circulation of scleroderma autoantibodies. Systemic scleroderma is a ...
... systemic sclerosis), including skin thickening, skin hardening, tight skin, itchy skin, fatigue, arthritis, and muscle pain. ... Because scleroderma (systemic sclerosis) primarily targets the skin, its most obvious symptoms show themselves as skin problems ... The lung disease often seen with scleroderma is interstitial lung disease (ILD). Pulmonary hypertension may also occur with ... It is important to identify and treat the organs affected with scleroderma. ...
Systemic scleroderma, also called diffuse scleroderma or systemic sclerosis, is an autoimmune disease of the connective tissue ... WrongDiagnosis , Diseases » Scleroderma » Prevalence Retrieved on Dec 13, 2009 *^ a b Systemic sclerosis (scleroderma) and ... There are two forms of scleroderma: localized and systemic. The localized (non-systemic) form affects the skin of only the face ... Masi AT (1980). "Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma ...
Treatment of Refractory Sever Systemic Scleroderma by Injection of Allogeneic Mesenchymal Stem Cells. *SYSTEMIC SCLERODERMA ... Cyclophosphamide and rATG With Hematopoietic Stem Cell Support in Systemic Scleroderma. *SYSTEMIC SCLERODERMA ... Neuropsychiatric Scleroderma Study: Systematic Evaluation of Neuropsychiatric Involvement in Systemic Sclerosis. *Systemic ... Trial of Topical C-82 in Systemic Sclerosis - A Phase I/II Biomarker and Safety Trial. *Systemic Scleroderma ...
... who were entered into the multicenter Scleroderma Criteria Cooperative Study (SCCS) during 1973-1977. At the end of the study ( ... We conducted followup of 264 patients with definite systemic sclerosis (SSc) ... Predictors of survival in systemic sclerosis (scleroderma) Arthritis Rheum. 1991 Apr;34(4):403-13. doi: 10.1002/art.1780340405 ... We conducted followup of 264 patients with definite systemic sclerosis (SSc) who were entered into the multicenter Scleroderma ...
... diffuse scleroderma, dSSc, limited scleroderma, lSSc, and CREST Syndrome. It can affect the skin, blood vessels, and internal ... Systemic sclerosis is also known as scleroderma, SSc, ... Also see Types of Scleroderma, and Systemic Scleroderma ... Systemic Scleroderma). There are several types of Systemic Scleroderma: CREST, Limited and Diffuse. Systemic scleroderma is ... Also see What is Scleroderma?, Types of Scleroderma and Systemic Symptoms). (PDF) Systemic Sclerosis. Systemic sclerosis (SSc) ...
HealingWell.com Forum , Diseases & Conditions , Lupus , Scleroderma and/or Systemic Sclerosis Select A Location. ****** Top of ... My diagnosis turned from Lupus (10/98) to Scleroderma in October of 2003. I did not notice a forum for us Scleroderma patients ... I also have scleroderma. A great site is sclero.org. But dont the people here really cant be beat!. emmie. Limited ... Oh also if you get a chance to look up choctaw indians and scleroderma together you might find some interesting info about how ...
Scleroderma or Progressive Systemic Sclerosis. Scleroderma or Progressive Systemic Sclerosis. - See: vascular malformations of ... Cold Hands Associated with Scleroderma. - Radiographs:. - diffuse subQ calcifications (calcinosis circumscripta) are seen;. - ...
Diffuse Scleroderma can cause Arrhythmias, Pericardial Effusion, and Heart Failure. Formation of scar tissue in the heart and ... Barb: Diffuse Systemic Scleroderma I was diagnosed in 1985 with systemic scleroderma and Raynauds. It has affected my ... Scleroderma Types Systemic Symptoms Cardiac. Systemic Scleroderma Cardiac (Heart) Involvement. Author: Shelley Ensz. ... Also see What is Scleroderma?, Types of Scleroderma, Symptoms of Scleroderma). Incidental significant arrhythmia in scleroderma ...
... systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage ... Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. The typical patient is a young or ... Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and ... The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin- ...
Systemic" by people in Harvard Catalyst Profiles by year, and whether "Scleroderma, Systemic" was a major or minor topic of ... "Scleroderma, Systemic" is a descriptor in the National Library of Medicines controlled vocabulary thesaurus, MeSH (Medical ... Below are the most recent publications written about "Scleroderma, Systemic" by people in Profiles. ... Below are MeSH descriptors whose meaning is more general than "Scleroderma, Systemic". ...
The etiology of the basic disease and pathogenesis of the systemic autoimmunity, fibrosis, and fibroproliferative vasculopathy ... Systemic sclerosis (SSc) is an acquired multiorgan connective tissue disease with variable mortality and morbidity dictated by ... scleroderma) Debendra Pattanaik1, Monica Brown2, Arnold E Postlethwaite11Division of Connective Tissue Diseases, Department of ... Abstract: Systemic sclerosis (SSc) is an acquired multiorgan connective tissue disease with variable mortality and morbidity ...
When scleroderma only affects the skin, it is considered localized. However, if it affects the skin and internal organs, it ... is viewed as systemic, also called Systemic Sclerosis (SSc). ... Localized scleroderma does NOT evolve into systemic scleroderma ... Localized scleroderma (also known as morphea or linear scleroderma). Two/thirds of those diagnosed with scleroderma have the ... It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal ...
... active life until Systemic Scleroderma ravaged his body and confined him to a wheelchair. Learn how adult stem cells saved ... "They told me I had systemic scleroderma," said Tony. "They treated me for 10 days and the day I checked out of the hospital, on ... Tony says since he suffered from systemic scleroderma, his entire body was attacked. "Youre just hard, youre hard as a rock ... Tony Underhill lived a full, active life until Systemic Scleroderma ravaged his body and confined him to a wheelchair. The ...
... also called scleroderma, is a chronic autoimmune disorder characterized by fibrosis of the skin and various organs. Early ... Systemic sclerosis (SSc), also called scleroderma, is a chronic autoimmune disorder characterized by fibrosis of the skin and ... ACR/EULAR Criteria for the Classification of Systemic Sclerodermaa. Category. Subitems. Score. ... Systemic Sclerosis - Scleroderma. ARUP Consult®. Retrieved December 01, 2020, https://arupconsult.com/content/systemic- ...
Treprostinil Iontophoresis in Systemic Sclerosis Digital UlcErations. A Proof of Concept Study. *Scleroderma, Systemic ... Also searched for Systemic sclerosis and Scleroderma. See Search Details. Applied Filters: Recruiting Not yet recruiting ... scleroderma, systemic sclerosis AND digital , Recruiting, Not yet recruiting, Available Studies (13 records) ... 13 Studies found for: scleroderma, systemic sclerosis AND digital , Recruiting, Not yet recruiting, Available Studies ...
Outcome and Prognostic Indicators in Early Scleroderma (Systemic Sclerosis) Annals of Internal Medicine; 118 (8): 602-609 ... D-Penicillamine Therapy in Progressive Systemic Sclerosis (Scleroderma): A Retrospective Analysis Annals of Internal Medicine; ... RENAL INVOLVEMENT IN PROGRESSIVE SYSTEMIC SCLEROSIS (SCLERODERMA)* ROBERT J. LEVINE, M.D.; BURIS R. BOSHELL, M.D. ... The Kidney in Progressive Systemic Sclerosis: A Prospective Study Annals of Internal Medicine; 89 (6): 881-887 ...
Scleroderma). Dietary changes can be generally helpful for people with this problem. ... Diet plan for patients of Systemic Sclerosis ( ... Diet Plan for Patients of Systemic Sclerosis (Scleroderma). ...
Scleroderma has been further subclassified into localized scleroderma (morphea), limited cutaneous systemic sclerosis ( ... Systemic Implications and Complications. Patients with limited cutaneous systemic sclerosis may experience myriad systemic ... Treatment options for limited cutaneous systemic sclerosis. Medical Treatment. Physical Modalities. Systemic Symptoms (fatigue ... Patients with polymyositis/systemic sclerosis overlap have been described with the typical features of systemic sclerosis in ...
Systemic sclerosis (SSc; scleroderma) is a chronic, multisystem disorder of connective tissue characterized by thickening and ... Ariane L. Herrick, Controversies on the Use of Steroids in Systemic Sclerosis, Journal of Scleroderma and Related Disorders, ... Canadian Scleroderma Research Ggroup. Search for more papers by this author*. Additional members of the Canadian Scleroderma ... Hypothesized etiologic factors for fatigue in systemic sclerosis (SSc; scleroderma). IL-1β = interleukin-1β; IL-6 = interleukin ...
Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the ... A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus ... Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or ... and Raynauds phenomenon was conducted in order to develop classification criteria for systemic sclerosis. ...
Diffuse systemic sclerosis (SSc; scleroderma) is a debilitating disease characterized by excessive dermal fibrosis with later ... A modified model of graft-versus-host-induced systemic sclerosis (scleroderma) exhibits all major aspects of the human disease. ... The present studies were undertaken to determine if alteration of the induction of GVH-induced scleroderma could result in a ... Diffuse Scleroderma - Genetic Alliance. *Systemic Scleroderma - Genetic Alliance. *Scleroderma - MedlinePlus Health Information ...
New ELISA blood test from Gemelli Biotech offers important insights to patients with Scleroderma and Systemic Sclerosis - read ... About scleroderma. Systemic Sclerosis and Scleroderma is a serious and sometimes fatal autoimmune disease that affects a ... New ELISA blood test from Gemelli Biotech offers important insights to patients with Scleroderma and Systemic Sclerosis. ... com/news-releases/new-elisa-blood-test-from-gemelli-biotech-offers-important-insights-to-patients-with-scleroderma-and-systemic ...
... or scleroderma) is long-term and causes thick, hard skin - possibly affecting your organs. Learn the symptoms, causes and ... What is systemic sclerosis (scleroderma)?. Systemic sclerosis is a rare condition that can cause peoples skin and the ... Systemic sclerosis is not to be confused with a separate condition called localised scleroderma, which just affects the skin. ... Living with systemic sclerosis. For some of the symptoms of systemic sclerosis you will need to take drugs. However, a healthy ...
To determine the efficacy of immunesuppressive treatment over 1 year in early diffuse cutaneous systemic sclerosis (dcSSc). ... Pope, J. (2011) Measures of Systemic Sclerosis (Scleroderma): Health Assessment Questionnaire (HAQ) and Scleroderma HAQ (SHAQ ... 2014) Effects and Safety of Rituximab in Systemic Sclerosis: An Analysis from the European Scleroderma Trial and Research ( ... Sekhon, S. and Pope, J., Canadian Scleroderma Research Group and Baron M. (2010) The Minimally Important Difference in Clinical ...
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Home › Forums › Personal History and Progress Threads › Diffuse Systemic scleroderma with severe pulmonary fibrosis ... In the autumn 2001 I was diagnosed with Diffuse Systemic Scleroderma[/b]. My symptoms started the same year in spring. First I ... From there till the final conclusion, that my disease is scleroderma, there were a lot of testing and a lot of waiting. In the ...
Systemic sclerosis , Healthy Control Subjects , Circulation Disorders , Raynauds Disease , Undifferentiated Connective Tissue ... Metabolomic Analysis of Systemic Sclerosis Brief description of study. Currently investigators do not have diagnostic and ...
  • Approximately 15 percent to 25 percent of people with features of systemic scleroderma also have signs and symptoms of another condition that affects connective tissue, such as polymyositis, dermatomyositis, rheumatoid arthritis , Sjögren syndrome , or systemic lupus erythematosus . (medlineplus.gov)
  • CREST may occur alone, or in combination with any other form of Scleroderma (or even other autoimmune diseases) such as CREST with Limited Scleroderma, or CREST with Lupus. (sclero.org)
  • If a scleroderma patient also has any other autoimmune disease (such as lupus, rheumatoid arthritis, etc.) it is referred to as overlap . (sclero.org)
  • My diagnosis turned from Lupus (10/98) to Scleroderma in October of 2003. (healingwell.com)
  • Dx lupus, scleroderma, pos. (healingwell.com)
  • For this Review, we systematically collated information on the cardiac phenotype in the most common inducible, spontaneous and engineered mouse models of systemic lupus erythematosus, rheumatoid arthritis and systemic sclerosis. (sclero.org)
  • Carter V, LaCava J, Taylor MS, Liang SY, Mustelin C, Ukadike KC, Bengtsson A, Lood C, Mustelin T. High Prevalence and Disease Correlation of Autoantibodies Against p40 Encoded by Long Interspersed Nuclear Elements in Systemic Lupus Erythematosus. (harvard.edu)
  • A multicenter, ongoing study of early-diagnosed cases of systemic sclerosis and comparison patients with systemic lupus erythematosus, polymyositis/dermatomyositis, and Raynaud's phenomenon was conducted in order to develop classification criteria for systemic sclerosis. (nih.gov)
  • Respiratory function in systemic lupus erythematosus, scleroderma, and rheumatoid arthritis. (bmj.com)
  • En s k rastlanan antikor IgG anti-β2 GPI idi (%37,5), bunu s ras yla antikardiyolipinler (%17,5) ve lupus antikoag lan (%5) izlemekteydi. (tjh.com.tr)
  • ICs were purified from the sera of SSc patients bearing different autoantibody specificities (antibodies against DNA topoisomerase I, centromeric proteins, RNA polymerase, and Th/To), patients with systemic lupus erythematosus (SLE) and primary anti-phospholipid syndrome (PAPS), or healthy controls (NHS) using polyethylene glycol precipitation. (biomedcentral.com)
  • Widespread scleroderma can also occur in association with other autoimmune diseases, including systemic lupus erythematosus and polymyositis . (rareguru.com)
  • These figures indicate that SScl is over twice as lethal as systemic lupus erythematosus (SLE). (houstonrheumatology.com)
  • Systemic lupus erythematosus (lupus) is a chronic autoimmune disease characterized by inflammation, pain and swelling. (childrens.com)
  • In 15 to 20 percent of cases, a person who has scleroderma also has symptoms of another autoimmune disease, such as rheumatoid arthritis, lupus or Sjogren's syndrome. (mayoclinic.org)
  • Percutaneous coronary intervention (PCI) outcomes in patients with rheumatoid arthritis, systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). (sclero.org)
  • If someone in your family has an autoimmune disease - such as lupus, rheumatoid arthritis or Hashimoto's disease - you have an increased risk of developing limited scleroderma. (mayoclinic.org)
  • Comparatively, patients with systemic lupus erythematosus (a chronic, autoimmune connective tissue disease) are at a much lower risk of developing PAH - around 17% of these patients develop PAH, according to Dr. Faber. (pulmonaryhypertensionnews.com)
  • We investigated killer immunoglobulin-like receptors (KIRs) and the human leukocyte antigen (HLA)-C ligands for the corresponding inhibitory KIRs in Caucasian patients, 304 with systemic lupus erythematosus (SLE) and 90 with scleroderma [or progressive systemic sclerosis (PSS)] compared with 416 Caucasian controls. (cdc.gov)
  • Disparate distribution of activating and inhibitory killer cell immunoglobulin-like receptor genes in patients with systemic lupus erythematosus. (cdc.gov)
  • Systemic lupus erythematosus (SLE) is a chronic life-long, autoimmune disease. (adam.com)
  • SLE is the most common type and is the type of lupus that can lead to serious systemic complications. (adam.com)
  • Systemic lupus erythematosus (SLE) is an autoimmune disorder. (adam.com)
  • The condition is also called systemic sclerosis because the fibrosis can affect organs other than the skin. (medlineplus.gov)
  • In the third type of systemic scleroderma, called systemic sclerosis sine scleroderma ("sine" means without in Latin), fibrosis affects one or more internal organs but not the skin. (medlineplus.gov)
  • However, if it affects the skin and internal organs, it is viewed as "systemic," also called Systemic Sclerosis (SSc). (hss.edu)
  • Systemic scleroderma is also called systemic sclerosis. (umm.edu)
  • In one type of scleroderma, called systemic sclerosis , you can also get hardening of some of your internal organs. (almondsburysurgery.nhs.uk)
  • Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers. (wikipedia.org)
  • The first symptom of scleroderma is frequently the development of the Raynaud's phenomenon, which is when the fingers and/or toes change color and become numb when exposed to the cold. (hss.edu)
  • Scleroderma has been further subclassified into localized scleroderma (morphea), limited cutaneous systemic sclerosis (previously CREST syndrome, standing for calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) and diffuse cutaneous systemic sclerosis. (oncologynurseadvisor.com)
  • Patients with early limited cutaneous systemic sclerosis may present with swollen erythematous hands and Raynaud's phenomenon. (oncologynurseadvisor.com)
  • Physical examination of a patient with limited systemic sclerosis may reveal mat-like telangiectasias, sclerosis of the peri-oral skin with a decrease in the oral aperture, sclerosis of the skin distal to the elbows and knees with pigmentary abnormalities, Raynaud's phenomenon with digital pulp ulceration or pitting ( Figure 1 ), sclerodactyly, nail fold capillary changes (capillary loop dilation, hemorrhage, or capillary drop-out), and cutaneous calcinosis. (oncologynurseadvisor.com)
  • Most people with systemic sclerosis develop an unusual sensitivity to the cold, known as Raynaud's phenomenon . (versusarthritis.org)
  • Many people have Raynaud's without ever having systemic sclerosis. (versusarthritis.org)
  • However, most people with systemic sclerosis will have symptoms of Raynaud's at some time during their illness. (versusarthritis.org)
  • Raynaud's is often the first sign that someone has systemic sclerosis. (versusarthritis.org)
  • Pope, J. (2011) Measures of Systemic Sclerosis (Scleroderma): Health Assessment Questionnaire (HAQ) and Scleroderma HAQ (SHAQ), Physician-and Patient-Rated Global Assessments, Symptom Burden Index (SBI), University of California, Los Angeles, Scleroderma Clinical Trials Consortium Gastrointestinal Scale (UCLA SCTC GIT) 2.0, Baseline Dyspnea Index (BDI) and Transition Dyspnea Index (TDI) (Mahler's Index), Cambridge Pulmonary Hypertension Outcome Review (CAMPHOR), and Raynaud's Condition Score (RCS). (scirp.org)
  • Centromere antibodies are found in patients with a limited form of cutaneous systemic sclerosis, "CREST" syndrome, a complex of subcutaneous calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias. (specialtylabs.com)
  • To increase the awareness of Raynaud's and Scleroderma amongst healthcare professionals, Government and Statutory agencies and the general public. (diseaseinfosearch.org)
  • if you have Raynaud's phenomenon it does NOT necessarily mean that you will develop systemic sclerosis. (almondsburysurgery.nhs.uk)
  • Raynaud's phenomenon on its own is a common condition and most people who have Raynaud's phenomenon will not develop systemic sclerosis. (almondsburysurgery.nhs.uk)
  • What is Raynaud's & Scleroderma & Associated Vascular Disorders? (circulationfoundation.org.uk)
  • Although over 95% of patients with scleroderma have Raynaud's, the chances of someone with Raynaud's developing scleroderma is small - it is less than 2% women and 6% in men. (circulationfoundation.org.uk)
  • There is no evidence at present that either Raynaud's or scleroderma are directly inherited. (circulationfoundation.org.uk)
  • Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis of the skin and visceral tissues as well as a non-inflammatory vasculopathy manifesting as Raynaud's phenomenon, digital ulcerations, pulmonary arterial hypertension, and scleroderma renal crisis. (hindawi.com)
  • Ninety percent of scleroderma patients will also suffer from Raynaud's phenomenon, which causes the blood vessels in the hands and feet to contract in cold conditions causing pain and discoloration. (sclerodermanews.com)
  • One of the earliest signs of systemic scleroderma is Raynaud's disease, which causes the small blood vessels in your fingers and toes to contract in response to cold temperatures or emotional distress. (mayoclinic.org)
  • Raynaud's disease also occurs in people who don't have scleroderma. (mayoclinic.org)
  • The variety of Raynaud's disease that occurs with systemic scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores. (mayoclinic.org)
  • Raynaud's and Scleroderma Association: offers support, advice and information to sufferers of Raynaud's and Scleroderma, two disorders which commonly occur together and affect the extremities, usually the fingers and toes. (netdoctor.co.uk)
  • produce publications about Raynaud's and scleroderma. (netdoctor.co.uk)
  • The Association distributes books, leaflets and videos on Raynaud's, scleroderma and other associated conditions. (netdoctor.co.uk)
  • vascular domain (Raynaud's phenomenon, abnormal capillaroscopy with scleroderma pattern) and laboratory domain (antinuclear, anticentromere and antitopoisomerase-I antibodies). (ox.ac.uk)
  • Raynaud's phenomena is often the first sign of limited scleroderma, but many people who have Raynaud's never develop scleroderma. (mayoclinic.org)
  • Systemic scleroderma is an autoimmune disorder that affects the skin and internal organs. (medlineplus.gov)
  • In another type of systemic scleroderma, known as diffuse cutaneous systemic scleroderma, the fibrosis affects large areas of skin, including the torso and the upper arms and legs, and often involves internal organs. (medlineplus.gov)
  • Systemic scleroderma, or systemic sclerosis, is an autoimmune rheumatic disease characterised by excessive production and accumulation of collagen, called fibrosis, in the skin and internal organs and by injuries to small arteries. (wikipedia.org)
  • Systemic scleroderma may affect the skin, blood vessels, and/or internal organs. (sclero.org)
  • Systemic sclerosis (systemic scleroderma) is a chronic connective tissue disease of unknown etiology that causes widespread microvascular damage and excessive deposition of collagen in the skin and internal organs. (aafp.org)
  • scleroderma) is a debilitating disease characterized by excessive dermal fibrosis with later progression to internal organs. (nih.gov)
  • When systemic sclerosis causes connective tissue to harden this can stop muscles, blood vessels, joints and internal organs such as the lungs, working as well as they should. (versusarthritis.org)
  • Diffuse systemic sclerosis can affect skin all over the body and is more likely to affect the internal organs. (versusarthritis.org)
  • Apart from its effects on the skin, systemic scleroderma can also harden the internal organs. (uofmhealth.org)
  • Systemic scleroderma affects the skin, as well as blood vessels and internal organs. (familydoctor.org)
  • Sclerosis sine scleroderma-very rare form of scleroderma in which there are no skin manifestations, but the internal organs are affected. (epnet.com)
  • Systemic sclerosis (SSc) is a clinically heterogenous chronic fibrotic disease which affects skin and internal organs. (bu.edu)
  • Systemic scleroderma - Affects widespread areas of skin and/or internal organs, most often the lungs. (uvahealth.com)
  • Diffuse scleroderma comes on more suddenly and can progress to involve not only the skin but the internal organs. (uvahealth.com)
  • Scleroderma is an uncommon disease that causes hard, thickened areas of skin and may also cause problems with blood vessels and some internal organs in the body. (mydr.com.au)
  • When internal organs are affected, fluctuating symptomatology is expected because systemic sclerosis can lead to alterations in any organ of the body. (symptoma.com)
  • Scleroderma is a group of autoimmune diseases that cause the skin and connective tissues (tissues that support the skin and internal organs) to get thick and hard. (childrens.com)
  • In the more severe form, called diffuse scleroderma, wide areas of skin and internal organs such as the lungs, bowel heart and kidneys are affected. (circulationfoundation.org.uk)
  • But in many people, scleroderma also harms structures beyond the skin, such as blood vessels, internal organs and the digestive tract (systemic scleroderma). (mayoclinic.org)
  • For example, Choctaw Native Americans are more likely to develop the type of scleroderma that affects internal organs. (mayoclinic.org)
  • Systemic Scleroderma is a rare autoimmune disease that can invade internal organs caused by an over production of collagen that hardens internal and external healthy tissue. (helpsavemike.ca)
  • in systemic scleroderma, the internal organs are affected as well. (thefreedictionary.com)
  • Systemic sclerosis (SSc) is characterized by autoimmunity, microangiopathy, and fibrosis in skin and internal organs. (biomedcentral.com)
  • The systemic forms can affect any part of the body (skin, blood vessels, and internal organs. (taragana.net)
  • Psoriasis and concomitant fibrosing disorders: Lichen sclerosus, morphea, and systemic sclerosis. (sclero.org)
  • I got diagnosed with Coup De Sabre linear morphea/scleroderma. (healingwell.com)
  • Histopathology is not required for diagnosis, and does not differentiate morphea from limited cutaneous systemic sclerosis or from diffuse cutaneous systemic sclerosis. (oncologynurseadvisor.com)
  • The differential diagnosis of limited cutaneous systemic sclerosis includes diffuse cutaneous systemic sclerosis, generalized morphea, scleromyxedema, nephrogenic systemic fibrosis, eosinophilic fasciitis, and graft-versus-host disease. (oncologynurseadvisor.com)
  • Generalized morphea can be difficult to differentiate from limited cutaneous systemic sclerosis. (oncologynurseadvisor.com)
  • There are several subtypes of juvenile scleroderma -- linear scleroderma, circumscribed morphea, generalized morphea, scleroderma en coup de sabre, and deeper lesions such as eosinophilic fasciitis. (dukehealth.org)
  • There are two main forms of localized scleroderma: morphea and linear scleroderma. (umm.edu)
  • In morphea scleroderma, patches of hard skin form and can last for years. (umm.edu)
  • A group of conditions known as "localized" sclero-derma, which includes morphea, linear scleroderma, and en coup de sabre, is discussed in the chapter on Scleroderma Mimickers (Chap. 11). (elsevier.com)
  • Localized scleroderma (can be morphea or linea) - Usually affects only the skin in isolated parts of the body. (uvahealth.com)
  • The morphea type of scleroderma usually strikes people around 20-40 years old. (uvahealth.com)
  • Localised scleroderma may be called morphea (patches of hard skin) or linear (long streaks or lines of hard skin). (mydr.com.au)
  • Morphea is a plaque-like area of skin thickening that commonly involves the trunk or limbs, and linear scleroderma is a potentially disfiguring process that causes streaks or "dents" to appear in the skin. (houstonrheumatology.com)
  • A two-component therapeutic consisting of FCX-013 and veledimex for the treatment of localized scleroderma (or morphea). (clinicaltrials.gov)
  • Fibrocell is developing a two-component therapeutic product for the treatment of moderate to severe localized scleroderma (morphea). (clinicaltrials.gov)
  • Localized scleroderma, known as morphea, may occur in patches or linear lesions, or it may be superficial. (thefreedictionary.com)
  • Guttate morphea (white-spot disease) is a manifestation of the superficial form of localized scleroderma and occurs mainly in women. (thefreedictionary.com)
  • In one type of systemic scleroderma, known as limited cutaneous systemic scleroderma, fibrosis usually affects only the hands, arms, and face. (medlineplus.gov)
  • Limited cutaneous systemic scleroderma used to be known as CREST syndrome, which is named for the common features of the condition: calcinosis, Raynaud phenomenon, esophageal motility dysfunction, sclerodactyly, and telangiectasia. (medlineplus.gov)
  • In diffuse cutaneous systemic scleroderma, the condition worsens quickly and organ damage occurs earlier than in other types of the condition. (medlineplus.gov)
  • Variations in the IRF5 gene are specifically associated with diffuse cutaneous systemic scleroderma, and a variation in the STAT4 gene is associated with limited cutaneous systemic scleroderma. (medlineplus.gov)
  • A 65-year-old woman with a 35-year history of limited cutaneous systemic scleroderma was admitted to our hospital complaining of a 3-month history of progressive dyspnoea on exertion. (bmj.com)
  • Hearing loss in diffuse cutaneous systemic scleroderma. (bvsalud.org)
  • The risk of renal damage from scleroderma renal crisis can be lessened by early detection, prompt initiation of angiotensin-converting enzyme inhibitor therapy, and avoidance of high-dose corticosteroids. (aafp.org)
  • Initiation and continuation of angiotensin-converting enzyme inhibitors are recommended in patients with scleroderma renal crisis, even in the presence of elevated creatinine levels. (aafp.org)
  • Although patients in each subset of disease share many comorbidities, patients with limited cutaneous systemic sclerosis have a better prognosis than patients with diffuse cutaneous systemic sclerosis and are less likely to suffer from renal crisis. (oncologynurseadvisor.com)
  • Recurring posterior reversible encephalopathy syndrome in a patient with polymyositis/systemic sclerosis overlap syndrome triggered by scleroderma renal crisis. (thefreelibrary.com)
  • Here we present a case with polymyositis (PM)/SSc overlap syndrome with scleroderma renal crisis (SRC) who developed PRES. (thefreelibrary.com)
  • Other clinical manifestations of SSc acknowledge a vascular etiology: pulmonary arterial hypertension (PAH), scleroderma renal crisis (SRC), and gastric antral vascular ectasia (GAVE) are underpinned by fibroproliferative microangiopathy [ 3 ]. (biomedcentral.com)
  • How is a scleroderma renal crisis treated? (medscape.com)
  • Patients with diffuse, rapidly progressive skin involvement have the highest risk of developing scleroderma renal crisis. (medscape.com)
  • Renal crisis occurs in about 10% of all patients with systemic sclerosis. (medscape.com)
  • Scleroderma renal crisis that is not treated promptly and aggressively invariably leads to renal failure requiring dialysis or renal transplantation, or even death. (medscape.com)
  • High doses of corticosteroids should be avoided in patients with systemic sclerosis owing to an increased risk of developing renal crisis. (medscape.com)
  • Vascular manifestations including scleroderma renal crisis and pulmonary arterial hypertension should be considered relative contraindications against pregnancy due to the high associations of both maternal and fetal morbidity and mortality. (hindawi.com)
  • The frequency of deaths due to renal crisis significantly decreased (mainly due to effectiveness of ACE Inhibitors), from 42% to 6% of scleroderma-related deaths (p 0.001), whereas the proportion of patients with scleroderma who died of pulmonary fibrosis increased (due to lack of significant treatment) from 6% to 33% (p 0.001). (clinicaltrials.gov)
  • The signs and symptoms of systemic scleroderma usually begin with episodes of Raynaud phenomenon , which can occur weeks to years before fibrosis. (medlineplus.gov)
  • Another early sign of systemic scleroderma is puffy or swollen hands before thickening and hardening of the skin due to fibrosis. (medlineplus.gov)
  • As scleroderma progresses, esophageal involvement from abnormalities in decreased motility may worsen due to progressive fibrosis (scarring). (wikipedia.org)
  • Incidental significant arrhythmia in scleroderma (SSC) associates with cardiac magnetic resonance measure of fibrosis and hs-TnI and NT-proBNP. (sclero.org)
  • Systemic sclerosis (systemic scleroderma) is a connective tissue disease associated with autoimmunity, vasculopathy, and fibrosis. (aafp.org)
  • The etiology of the basic disease and pathogenesis of the systemic autoimmunity, fibrosis, and fibroproliferative vasculopathy are unknown and debated. (dovepress.com)
  • Systemic sclerosis (SSc), also called scleroderma, is a chronic autoimmune disorder characterized by fibrosis of the skin and various organs. (arupconsult.com)
  • Preliminary criteria are proposed namely, the finding of either the sole major criterion, i.e., proximal scleroderma, or two or more of the minor criteria, i.e., 1) sclerodactyly, 2) digital pitting scars of fingertips or loss of substance of the distal finger pad, and 3) bilateral basilar pulmonary fibrosis. (nih.gov)
  • Lung fibrosis affects many scleroderma patients and we were encouraged to see that the drug appeared to prevent decline in lung function," Khanna says. (uofmhealth.org)
  • Systemic sclerosis (SSc) is an autoimmune disease characterized by vascular abnormalities, and cutaneous and visceral fibrosis. (specialtylabs.com)
  • CD19 regulates skin and lung fibrosis via Toll-like receptor signaling in a model of bleomycin-induced scleroderma. (semanticscholar.org)
  • The fatigue that accompanies scleroderma is not only an unwelcome, relentless symptom which is difficult to control and manage but can also quite often be overlooked, with the initial management of the disease focusing on the more important (quite rightly), life threatening skin and organ fibrosis progression. (sclerodermanews.com)
  • Fibrosis and immune dysregulation in systemic sclerosis. (medscape.com)
  • Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by progressive fibrosis of the skin and visceral tissues as well as a noninflammatory vasculopathy. (hindawi.com)
  • Lung involvement in systemic sclerosis patients can lead to chronic lung conditions such as pulmonary fibrosis and pulmonary hypertension . (sclerodermanews.com)
  • Recent findings implicate these molecules as pathophysiological players in scleroderma skin and lung fibrosis. (springer.com)
  • Clinical appearance of acrosclerotic piece-meal necrosis of the thumb in a patient with systemic sclerosis. (wikipedia.org)
  • Early- versus late-onset systemic sclerosis: differences in clinical presentation and outcome in 1037 patients. (sclero.org)
  • Cardiac involvement (CI) in systemic sclerosis (SSc) : differences between clinical subsets and influence on survival. (sclero.org)
  • Patients with systemic sclerosis can be classified into two distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and survival. (aafp.org)
  • Systemic sclerosis (SSc) is an acquired multiorgan connective tissue disease with variable mortality and morbidity dictated by clinical subset type. (dovepress.com)
  • What is the role of laboratory testing versus clinical findings in diagnosing systemic sclerosis? (arupconsult.com)
  • Diagnosis of systemic sclerosis (SSc) involves a combination of clinical findings and autoantibody test results. (arupconsult.com)
  • 2012) Patterns and Predictors of Change in Outcome Measures in Clinical Trials in Scleroderma: An Individual Patient Meta-Analysis of 629 Subjects with Diffuse Cutaneous Systemic Sclerosis. (scirp.org)
  • 2009) Clinical Correlates of Self-Reported Physical Health Status in Systemic Sclerosis. (scirp.org)
  • Sekhon, S. and Pope, J., Canadian Scleroderma Research Group and Baron M. (2010) The Minimally Important Difference in Clinical Practice for Patient-Centered Outcomes Including Health Assessment Questionnaire, Fatigue, Pain, Sleep, Global Visual Analog Scale, and SF-36 in Scleroderma. (scirp.org)
  • Lawrence, E., Pope, J., Al-Zahraly, Z., Lalani, S. and Baron, M. (2009) The Relationship between Changes in Self-Reported Disability (Measured by the Health Assessment Questionnaire-HAQ) in Scleroderma and Improvement of Disease Status in Clinical Practice. (scirp.org)
  • Although there are currently no approved treatments for the estimated 125,000 people in the United States with scleroderma, Khanna and his team have been spearheading new clinical trials that could help shape the future of care. (uofmhealth.org)
  • It's a primary outcome measure in systemic sclerosis clinical trials. (uofmhealth.org)
  • Which is why he led the development of the American College of Rheumatology Provisional Composite Response Index for Clinical Trials in Early Diffuse Cutaneous Systemic Sclerosis , or ACR CRISS , in 2016. (uofmhealth.org)
  • The new measure was recently endorsed by the American College of Rheumatology Board of Directors at its 2018 annual meeting to be used in future systemic sclerosis clinical trials. (uofmhealth.org)
  • To determine the frequency and suppressive capacity of regulatory T cells (T(reg)) and their association with clinical parameters in patients with systemic scleroderma (SSc). (nih.gov)
  • Finding the right clinical trial for Systemic Scleroderma can be challenging. (diseaseinfosearch.org)
  • Clinical and serological features of systemic sclerosis in a multicenter African American cohort: Analysis of the genome research in African American scleroderma patients clinical database. (diseaseinfosearch.org)
  • The aim of our study was to search for APLs in patients affected by systemic scleroderma and to evaluate their involvement in the clinical manifestations of this disease. (tjh.com.tr)
  • Objective: To determine the relationships between systemic sclerosis (SSc)-related autoantibodies, as well as their clinical associations, in a well-characterized Australian patient cohort. (edu.au)
  • The Scleroderma Foundation in no way endorses any drugs, treatments, clinical trials, or studies reported in the eLetter. (scleroderma.org)
  • Because systemic scleroderma is not caused by a mutation in any one specific gene, clinical genetic testing to confirm a diagnosis or identify a "carrier" is not currently available. (rareguru.com)
  • You can also view a list of clinical trials involving people with systemic scleroderma on ClinicalTrials.gov . (rareguru.com)
  • Patients with diffuse cutaneous systemic sclerosis (dcSSc) have distinct clinical manifestations, usually with more aggressive organ involvement and highermortality than limited cutaneous systemic sclerosis (lcSSC). (bvsalud.org)
  • Cardiac magnetic resonance predicts ventricular arrhythmias in scleroderma (SSc): the Scleroderma Arrhythmia Clinical Utility Study (SanCtUS). (sclero.org)
  • Eight hundred thirty-seven women with a clinical diagnosis of SSc, recruited a s a volunteer sample from 3 university-based, tertiary care scleroderma clinical research centers, and 2,507 race-matched local control women, recruited by the technique of random-digit-dialing and frequency-matched on age, completed a questionnaire providing data on history of augmentation mammoplasty, including possible complications of the procedure. (docme.su)
  • The clinical significance of ANCA in [systemic sclerosis (SSc)] patients who do not manifest [ANCA-associated vasculitis] is controversial. (healio.com)
  • Aims and Methods: The baseline data of all SSc patients entered in the EULAR Scleroderma Trials and Research (EUSTAR) database up to April 2007 were analysed for geographical differences with regard to organ involvement, and geographical clusters with regard to clinical subsets (diffuse vs limited SSc) and autoantibodies (anticentromere vs anti-Scl70). (elsevier.com)
  • This progression from scleroderma into pulmonary arterial hypertension was recently discussed by renowned PH expert Harrison Farber, MD , at the American College of Rheumatology's State-of-the-Art Clinical Symposium in Chicago. (pulmonaryhypertensionnews.com)
  • According to Dr. Farber, people with scleroderma are at the highest risk of developing PAH, which is why clinical trials involving PAH patients typically recruit around 60% (systemic) scleroderma-affected patients. (pulmonaryhypertensionnews.com)
  • Although no disease-modifying therapy has been proven effective, complications of systemic sclerosis are treatable, and interventions for organ-specific manifestations have improved substantially. (aafp.org)
  • LOS ANGELES , Jan. 9, 2019 /PRNewswire/ -- The new blood test, sclero-smart™ , measures levels of anti-vinculin, recently found to be elevated in patients with scleroderma and associated with gastrointestinal complications. (biospace.com)
  • Given the known link between anti-vinculin and intestinal neuropathy, anti-vinculin has substantial potential to serve as a marker for GI complications in scleroderma patients. (biospace.com)
  • Therefore, the measurement of anti-vinculin can be an important test to help rheumatologists identify the risk of GI or vascular complications in scleroderma patients. (biospace.com)
  • No statistically significant association of positive antiphospholipid-related tests to any of the scleroderma complications could be demonstrated. (tjh.com.tr)
  • It would be useful to follow a cohort of patients affected by systemic scleroderma in order to monitor vascular complications following confirmation of the presence of antiphospholipid syndrome. (tjh.com.tr)
  • What are the possible complications of scleroderma? (familydoctor.org)
  • Its course is very slow, and it rarely, if ever, spreads throughout the body (becomes systemic) or causes serious complications. (umm.edu)
  • Complications of diffuse scleroderma can affect virtually every system of the body. (uvahealth.com)
  • Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease. (aappublications.org)
  • Complications with systemic sclerosis (scleroderma) heart involvement include atrioventricular heart block, cardiogenic shock, myocardial infarction, and skeletal myopathy. (sclero.org)
  • Current treatment of systemic sclerosis is directed toward managing complications and providing symptomatic relief. (medscape.com)
  • Early detection of limited scleroderma can help prevent serious complications. (mayoclinic.org)
  • Patients with scleroderma who do not have any symptoms of cardio-pulmonary complications have a better prognosis, as do patients affected by both ILD and scleroderma. (pulmonaryhypertensionnews.com)
  • Some of the other risk factors in people with scleroderma pertaining to development of PAH and related complications include increasing age, pericardial effusion and liver disease. (pulmonaryhypertensionnews.com)
  • There are two major subgroups of systemic sclerosis based on the extent of skin involvement: limited and diffuse. (wikipedia.org)
  • The severity of these symptoms varies greatly among patients: Some having scleroderma of only a limited area of the skin (such as the fingers) and little involvement of the underlying tissue, while others have progressive skin involvement. (wikipedia.org)
  • Some lSSc patients who have no cutaneous involvement but common SSc nail fold capillaroscopy abnormalities, specific antinuclear antibodies, and visceral involvement are sometimes called SSc sine scleroderma. (sclero.org)
  • Limited Scleroderma is when skin involvement is limited to the hands (although the face and neck may also be involved. (sclero.org)
  • Heart involvement in systemic sclerosis. (sclero.org)
  • Primary and secondary heart involvement in systemic sclerosis are important mortality predictors and left ventricular diastolic impairment was the most frequent echographic abnormality in our study. (sclero.org)
  • Silent cardiovascular involvement in patients with diffuse scleroderma: A controlled cross-sectional study. (sclero.org)
  • The third and less prevalent subtype is SSc sine scleroderma, or SSc without hard skin, meaning a person has the internal organ manifestations and perhaps laboratory evidence for the condition, but no skin involvement. (hss.edu)
  • Renal involvement in progressive systemic sclerosis has been recognized for many years. (annals.org)
  • For patients affected by systemic scleroderma (also known as systemic sclerosis), a chronic autoimmune disease marked by hardening of the skin and internal organ involvement, the need for treatments is well understood. (uofmhealth.org)
  • Although involvement of the heart and the occurrence of arrhythmias in systemic scleroderma is well documented, 1,2,8.4,5,6 only a few cases of complete heart block in generalised scleroderma have been reported in the literature. (semanticscholar.org)
  • Non-invasive assessment of early cardiac involvement in systemic sclerosis. (semanticscholar.org)
  • A physical exam of your child's skin involvement will help to diagnose the type of juvenile localized scleroderma affecting your child. (dukehealth.org)
  • While there is no cure for juvenile localized scleroderma, your child's pediatric rheumatologist will recommend an appropriate treatment that is dependent on the type and size of the skin involvement. (dukehealth.org)
  • Almost all systemic sclerosis patients will experience hardening of the skin to some extent, many will also have internal organ involvement. (sclerodermanews.com)
  • Diffuse scleroderma patients can experience skin hardening on any part of their body and are more likely to suffer from organ involvement, particularly the kidneys, lungs, and heart. (sclerodermanews.com)
  • Limited scleroderma patients will generally have less skin hardening and are less likely to suffer from organ involvement. (sclerodermanews.com)
  • Involvement may be diffuse (systemic sclerosis) or localized to the skin (localized scleroderma). (aappublications.org)
  • The goal of this article is to review recent studies on the involvement of SIRTs in scleroderma from the perspective of aging-related molecular mechanisms. (springer.com)
  • Background: Atrophy of the smooth muscle layers of the muscularis propria characterises oesophageal involvement in systemic sclerosis (scleroderma). (elsevier.com)
  • For patients with Systemic Sclerosis (SSc), cardiac involvement (CI) is directly caused by myocardial fibrosisor ischemia or is secondary to pulmonary arterial hypertension1 Here we present a similar case of a femalepatient aged 45 yr old female who presented with complaint of recurrent syncopal attacks, and difficulty inswallowing food and liquids. (who.int)
  • Jafarinejad-Farsangi S, Gharibdoost F, Farazmand A, Kavosi H, Jamshidi A, Karimizadeh E, Noorbakhsh F, Mahmoudi M. MicroRNA-21 and microRNA-29a modulate the expression of collagen in dermal fibroblasts of patients with systemic sclerosis. (harvard.edu)
  • Scleroderma causes your body to produce too much collagen . (familydoctor.org)
  • Overproduction of collagen and other connective tissue proteins are the main features of scleroderma. (uvahealth.com)
  • People with scleroderma have too much of a protein called collagen in their connective tissue, making it hard and tight. (mydr.com.au)
  • Scleroderma develops because of an over-production and build-up of collagen in the body. (mydr.com.au)
  • Systemic sclerosis or scleroderma is a rare autoimmune disease where the body generates too much collagen. (sclerodermanews.com)
  • Scleroderma is a rare disease of unknown etiology in which increased collagen deposition occurs and results in dermal thickening. (aappublications.org)
  • Scleroderma results from an overproduction and accumulation of collagen in body tissues. (mayoclinic.org)
  • Smad7 constitutively formed a complex with the TGF-β receptors, and the inhibitory effect of Smad7 on the promoter activity of human α2(I) collagen and 3TP-lux was completely impaired in scleroderma fibroblasts. (jci.org)
  • Background: Systemic sclerosis (SSc) is a vasculopathy with increased tissue deposition of collagen. (elsevier.com)
  • And today, after undergoing an autologous adult stem-cell transplant 14 months ago to stop his advanced case of scleroderma, an incurable autoimmune disease that causes excess collagen to make skin tighten and feel hard and thick, George is able to pinch his pliable skin. (taragana.net)
  • Doppler echocardiography, pulmonary function testing, and high-resolution computed tomography of the chest should be performed at diagnosis of systemic sclerosis and at regular intervals thereafter. (aafp.org)
  • 12,000 human genes to characterize gene expression patterns in skin biopsies from individuals with a diagnosis of systemic sclerosis with diffuse scleroderma. (semanticscholar.org)
  • Preliminary criteria for the very early diagnosis of systemic sclerosis: results of a Delphi Consensus Study from EULAR Scleroderma Trials and Research Group. (ox.ac.uk)
  • OBJECTIVE: To identify a core set of preliminary items considered as important for the very early diagnosis of systemic sclerosis (SSc). (ox.ac.uk)
  • Linear scleroderma is more common in children. (familydoctor.org)
  • Linear scleroderma causes bands of hard skin across the face or on a single arm or leg. (umm.edu)
  • Linear scleroderma may also involve muscle or bone. (umm.edu)
  • Linear scleroderma often occurs in children. (uvahealth.com)
  • Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. (aappublications.org)
  • When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. (aappublications.org)
  • Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. (aappublications.org)
  • 1 Linear scleroderma frequently occurs on the limbs but also may develop in the frontoparietal area of the forehead and scalp. (aappublications.org)
  • Neurologic abnormalities have been described in association with linear scleroderma en coup de sabre. (aappublications.org)
  • Their cases illustrate the variability in neurologic manifestations of linear scleroderma en coup de sabre as well as the importance of a careful skin examination in patients with unexplained neurologic disease. (aappublications.org)
  • A diagnosis of linear scleroderma en coup de sabre was made based on the characteristic skin changes observed. (aappublications.org)
  • Linear scleroderma, marked by single or multiple linear lesions, occurs most frequently in children and is limited to the face or extremities. (thefreedictionary.com)
  • What type of scleroderma do I have? (familydoctor.org)
  • Duke pediatric rheumatologists identify the type of scleroderma affecting your child and use the latest therapies to reduce symptoms and minimize damage to skin, muscle, joints, and bones. (dukehealth.org)
  • Your child's pediatric rheumatologist may perform one or more of the following tests to diagnose the type of scleroderma affecting your child and to determine the most effective way to control the condition. (dukehealth.org)
  • Rarely, if this type of scleroderma affects children or young adults, it may interfere with growth and cause severe deformities in the arms and legs. (umm.edu)
  • This type of scleroderma usually only affects the skin and is considered the milder form of the disease. (mydr.com.au)
  • This type of scleroderma may affect the connective tissue throughout the body. (mydr.com.au)
  • In someone with the linear type of scleroderma, the hard areas of skin are in the shape of long streaks or lines, often starting on the arm, leg, neck or forehead. (mydr.com.au)
  • This type of scleroderma is more common in children. (almondsburysurgery.nhs.uk)
  • Signs and symptoms vary, depending on which type of scleroderma you have. (mayoclinic.org)
  • The number, location and size of the patches vary by type of scleroderma. (mayoclinic.org)
  • According to an abstract published recently by the American College of Rheumatology, up to 38% of people with scleroderma tested positive for elevated anti-vinculin antibodies, making anti-vinculin more prevalent in the scleroderma population than any other marker currently used for testing. (biospace.com)
  • Objective: To determine the efficacy of immunesuppressive treatment over 1 year in early diffuse cutaneous systemic sclerosis (dcSSc). (scirp.org)
  • Objectives: The rarity of early diffuse cutaneous systemic sclerosis (dcSSc) makes randomised controlled trials very difficult. (lu.se)
  • OBJECTIVES Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). (uzh.ch)
  • In the prospective Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3 comparison of autologous HSCT with 12 successive monthly intravenous pulses of cyclophosphamide in 156 patients with early diffuse cutaneous systemic sclerosis, HCST was associated with higher treatment-related mortality than in the first year after treatment. (medscape.com)
  • Systemic scleroderma is also known as systemic sclerosis (SSc). (sclero.org)
  • There are three types of systemic scleroderma, defined by the tissues affected in the disorder. (medlineplus.gov)
  • dSSC: Diffuse systemic sclerosis is one of the two more common types of systemic scleroderma. (symptoma.com)
  • In addition, people with systemic scleroderma often have open sores (ulcers) on their fingers, painful bumps under the skin (calcinosis), or small clusters of enlarged blood vessels just under the skin (telangiectasia). (medlineplus.gov)
  • seen in about 30% of people with systemic scleroderma). (mydr.com.au)
  • People with systemic scleroderma will often need a range of treatments and may be managed by several medical specialists, depending on what organ systems are affected. (mydr.com.au)
  • The combination of systemic scleroderma with other connective tissue abnormalities is known as scleroderma overlap syndrome. (medlineplus.gov)
  • Systemic scleroderma , also called diffuse scleroderma or systemic sclerosis , is an autoimmune disease of the connective tissue . (wikipedia.org)
  • Systemic sclerosis is a rare condition that can cause people's skin and the connective tissue inside the body to harden. (versusarthritis.org)
  • Systemic sclerosis can also cause connective tissue inside the body to become thick and hard. (versusarthritis.org)
  • The following information is about the condition systemic sclerosis, in which skin and connective tissue inside the body are affected. (versusarthritis.org)
  • Scleroderma is a disease of the body's connective tissue. (epnet.com)
  • Scleroderma is a type of connective tissue disease - connective tissue is the mesh of tissue that connects and supports the muscles, joints, organs and skin. (mydr.com.au)
  • Systemic scleroderma is a connective tissue disorder , which affects the skin, vessels and organs such as the heart, digestive tract or lungs. (symptoma.com)
  • In systemic sclerosis, the immune system damages the connective tissue cells, which are found in many parts of the body. (almondsburysurgery.nhs.uk)
  • So, systemic sclerosis is also classed as a connective tissue disease. (almondsburysurgery.nhs.uk)
  • Systemic scleroderma is characterized mainly by the disruption of microcirculation and of the functioning of the connective tissue as a whole, particularly of fibrogenesis. (thefreedictionary.com)
  • Scleroderma is treated by eliminating the disease's causative factors and by administering corticosteroids, vasodilators, and agents acting on the permeability of vascular connective tissue barriers. (thefreedictionary.com)
  • The lung disease often seen with scleroderma is interstitial lung disease (ILD) . (nationaljewish.org)
  • Treating active interstitial lung disease with oral cyclophosphamide (Cytoxan) for one year modestly improves lung function, dyspnea, skin thickening, and health-related quality of life in patients with systemic sclerosis. (aafp.org)
  • However, patients with limited cutaneous systemic sclerosis are more likely to suffer from isolated pulmonary artery hypertension (PAH ,ie, pulmonary hypertension not associated with interstitial lung disease [ILD] or left heart failure) than patients with diffuse cutaneous systemic sclerosis, and are at high risk of developing ILD. (oncologynurseadvisor.com)
  • Anti-Th/To antibodies are primarily associated with localized cutaneous scleroderma, and associated with pericarditis, interstitial lung disease and a high frequency of "intrinsic" pulmonary hypertension, and a poorer prognosis. (specialtylabs.com)
  • Anti-neutrophil cytoplasmic antibodies are linked to an increased prevalence of interstitial lung disease and pulmonary embolism in systemic sclerosis, according to data published in Arthritis Research & Therapy . (healio.com)
  • However, despite progress, PAH and interstitial lung disease (ILD) are still the leading causes of death in patients with scleroderma. (pulmonaryhypertensionnews.com)
  • The localized (non-systemic) form affects the skin of only the face, hands, and feet. (wikipedia.org)
  • When scleroderma only affects the skin, it is considered "localized. (hss.edu)
  • Limited systemic sclerosis is a milder form that only affects the skin on the face, lower arms, hands, lower legs and feet. (versusarthritis.org)
  • Systemic sclerosis is not to be confused with a separate condition called localised scleroderma, which just affects the skin. (versusarthritis.org)
  • Localized scleroderma affects the skin. (familydoctor.org)
  • Limited scleroderma also can affect your lungs, esophagus , and blood vessels. (familydoctor.org)
  • Systemic sclerosis might be a vascular disease, which begins by targeting blood vessels. (sclero.org)
  • The remaining eight patients were classified as having suspected systemic sclerosis (suspSSc), with Raynaud phenomenon and sclerodactyly (Supplementary Table 1 ). (biomedcentral.com)
  • Lungs Some impairment in lung function is almost universally seen in patients with diffuse scleroderma on pulmonary function testing, but it does not necessarily cause symptoms, such as shortness of breath. (wikipedia.org)
  • This differentiates them from patients with diffuse cutaneous systemic sclerosis, who present with proximal (above the knee and elbow) skin thickening. (oncologynurseadvisor.com)
  • Although anti-topoisomerase antibodies (otherwise known as anti-Scl-70 antibodies) are more commonly seen in patients with diffuse cutaneous systemic sclerosis, they can be seen in up to 25% of patients with limited cutaneous systemic sclerosis. (oncologynurseadvisor.com)
  • Cumberland Pharmaceuticals is conducting a research study to determine whether a drug product called ifetroban will prevent cardiomyopathy in patients with diffuse scleroderma or pulmonary arterial hypertension associated with scleroderma. (scleroderma.org)
  • In the Phase III trial, focuSSced , patients with active systemic sclerosis - the most serious form of scleroderma - were randomly assigned to receive either weekly double-blind injections of tocilizumab (an immunosuppressive drug mainly for treating rheumatoid arthritis), or a placebo for 48 weeks. (uofmhealth.org)
  • Therapeutic mud applications may benefit cardiovascular patients with rheumatoid arthritis, osteoarthrosis and systemic scleroderma. (greenmedinfo.com)
  • His most recent study, "Association of Defective Regulation of Autoreactive Interleukin-6-Producing Transitional B Lymphocytes with Disease in Patients with Systemic Sclerosis," appears in the March 2018 edition of Arthritis & Rheumatology . (the-rheumatologist.org)
  • As members of the Childhood Arthritis and Rheumatology Research Alliance , we helped develop and are now testing a standard approach to treating juvenile localized scleroderma. (dukehealth.org)
  • van den Hoogen F, Khanna D, Fransen J, Johnson SR, Baron M, Tyndall A. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. (medscape.com)
  • As we know, the [American College of Rheumatology] recommends that anyone who is newly diagnosed with scleroderma and / or is diagnosed should have an [echocardiogram], or sooner if their symptoms change," Farber said. (pulmonaryhypertensionnews.com)
  • Learn more about risk factors, screening tests, and treatment options for PAH in a special article that appeared in the Fall 2017 edition of Scleroderma Voice magazine, the member magazine of the Scleroderma Foundation. (scleroderma.org)
  • Philip Clements , M.D., M.P.H., recorded at the 2017 Scleroderma Foundation's National Patient Education Conference discussing dry eyes and dry mouth, which are frequently reported by patients with systemic sclerosis. (scleroderma.org)
  • Learn more about risk factors, screening tests, and treatment options for PAH in a special reprint from the Fall 2017 edition of Scleroderma Voice magazine, the member magazine of the Scleroderma Foundation. (scleroderma.org)
  • Our generous donors made it possible for 55 individuals affected by scleroderma to attend our 2017 National Patient Education Conference , exceeding our goal of 50 scholarships. (scleroderma.org)
  • A more quickly progressing form of scleroderma that causes the skin to thicken throughout the body. (epnet.com)
  • Certain categories of this form of scleroderma are more serious and can be fatal. (uvahealth.com)
  • Doppler myocardial imaging (DMI) can identify patients with a more diffuse and severe form of scleroderma, ideally before they develop severe vascular problems. (sclero.org)
  • Global Markets Direct's, 'Systemic Sclerosis (Scleroderma) - Pipeline Review, H2 2018', provides an overview of the Systemic Sclerosis (Scleroderma) pipeline landscape. (globalmarketsdirect.com)
  • Skin lesions observed in patients with systemic scleroderma change with the time and follow a definite course: they first appear as red-to-purple edematous lesions, then become hardened and ultimately transform into atrophic lesions. (symptoma.com)
  • CREST syndrome ( c alcinosis , R aynaud's phenomenon , e sophageal dysfunction, s clerodactyly , and t elangiectasia ) is associated with limited scleroderma. (wikipedia.org)
  • Limited Scleroderma (also called CREST Syndrome). (umm.edu)
  • Limited scleroderma, also known as CREST syndrome, is one subtype of scleroderma - a condition whose name means "hardened skin. (mayoclinic.org)
  • The systemic forms are also referred to as "systemic sclerosis" plus other terms such as diffuse, limited, CREST, and overlap. (taragana.net)
  • The systemic forms ( limited or diffuse cutaneous) involve those and, in addition, may progress to visceral organs , including the kidneys , heart , lungs , and gastrointestinal tract . (wikipedia.org)
  • Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. (mayoclinic.org)
  • Limited scleroderma can also affect your digestive tract, heart, lungs or kidneys. (mayoclinic.org)
  • Autoantibodies to PM/Scl, the human exosome complex, are found in polymyositis/scleroderma overlap syndromes. (specialtylabs.com)
  • The disease is associated with autoantibodies … so in scleroderma patients, the immune system produces antibodies that don't kill pathogens but attack self-proteins. (the-rheumatologist.org)
  • Consistently with their diagnostic and prognostic value, autoantibodies specific for systemic sclerosis (SSc) embedded in immune complexes (ICs) elicited a pro-inflammatory and pro-fibrotic cascade in healthy skin fibroblasts, engaging Toll-like receptors (TLRs) via their nucleic acid components. (biomedcentral.com)
  • These data provide the first demonstration of the pathogenicity of ICs from scleroderma patients with different autoantibodies on the endothelium. (biomedcentral.com)
  • Objective: Antiphospholipid antibodies (APLs) could be associated with an increased risk of vascular pathologies in systemic scleroderma. (tjh.com.tr)
  • Review: evidence that systemic sclerosis is a vascular disease. (medscape.com)
  • Role of Endothelial to Mesenchymal Transition in the Pathogenesis of the Vascular Alterations in Systemic Sclerosis. (medscape.com)
  • Vascular disease in systemic sclerosis is a major cause of morbidity and mortality among nonpregnant patients with SSc and is even a bigger concern in the pregnant SSc patient, as the underlying vasculopathy may prevent the required hemodynamic changes necessary to support a growing pregnancy. (hindawi.com)
  • Vascular disease in systemic sclerosis is a major cause of morbidity and mortality among non-pregnant patients with SSc. (hindawi.com)
  • Although not definitive, it has been suggested that an initial fall in systemic vascular resistance leads to the other well-established vascular and hemodynamic changes of pregnancies [ 3 ]. (hindawi.com)
  • Systemic sclerosis (SSc, scleroderma) is a multisystem autoimmune rheumatic disease that causes in﫿ammation, vascular damage and 﫿brosis. (clinicaltrials.gov)
  • Antiphospholid antibodies are frequently found in systemic scleroderma patients. (sclero.org)
  • The majority of patients with limited cutaneous systemic sclerosis will have positive anti-nuclear-antibodies. (oncologynurseadvisor.com)
  • Forty percent to 90% of patients with limited cutaneous systemic sclerosis will have positive anti-centromere antibodies. (oncologynurseadvisor.com)
  • Antibodies to three major components of U1-snRNP (snRNP RNP A, U1-snRNP RNP C, U1-snRNP RNP-70kd): U1- are associated with scleroderma and inflammatory myopathy overlap syndromes. (specialtylabs.com)
  • Limited systemic sclerosis can affect the lungs and the digestive system too. (versusarthritis.org)
  • This is because in patients with scleroderma, there is progressive blood vessel contraction (narrowing) that often leads to an increase in blood pressure in the lungs, which can in turn develop into PAH. (pulmonaryhypertensionnews.com)
  • Cardiac phenotype in mouse models of systemic autoimmunity. (sclero.org)
  • Systemic sclerosis (SSc) is a debilitating, chronic autoimmune disease that causes the body's immune system to attack its own tissue. (sclero.org)
  • Because systemic sclerosis (SSc) is a chronic illness, regular monitoring is needed to assess disease activity and progression. (arupconsult.com)
  • When scleroderma is chronic, treatment is carried out at sanatoriums and health resorts such as those located at Sochi, Piatigorsk, and Evpatoriia. (thefreedictionary.com)
  • Involves Raynaud phenomenon and some features of systemic sclerosis. (epnet.com)
  • Other symptoms include: In the skin, systemic sclerosis causes hardening and scarring. (wikipedia.org)
  • Calcinosis (deposition of calcium in lumps under the skin) is also common in systemic scleroderma, and is often seen near the elbows, knees, or other joints. (wikipedia.org)
  • Because scleroderma (systemic sclerosis) primarily targets the skin, its most obvious symptoms show themselves as skin problems. (nationaljewish.org)
  • Diffuse Scleroderma is when skin tightening also occurs above the wrists (or elbows, see below). (sclero.org)
  • Raynaud phenomenon and scleroderma (hardening of the skin) are hallmarks of the disease. (aafp.org)
  • The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA. (harvard.edu)
  • The term scleroderma comes from the Greek skleros , meaning hard, and derma, meaning skin. (hss.edu)
  • Almost all patients with Systemic Sclerosis (SSc) have thickening of their skin. (hss.edu)
  • It is possible to have Systemic Sclerosis without skin thickening (sine scleroderma), which is when a patient has the internal organ manifestations without the skin findings, but this is very rare. (hss.edu)
  • As stated previously, the physical examination finding that differentiates diffuse cutaneous systemic sclerosis from limited cutaneous systemic sclerosis is sclerosis of the skin proximal to the elbows and knees. (oncologynurseadvisor.com)
  • You can sometimes see signs of systemic sclerosis on the face, hands, feet and elsewhere around the body, as skin hardens and thickens. (versusarthritis.org)
  • The word scleroderma, which is an older term, specifically means hard skin. (versusarthritis.org)
  • Skin is one aspect of the disease, but scleroderma also affects other organs. (uofmhealth.org)
  • Red, shiny, tight skin on the hand due to scleroderma. (the-rheumatologist.org)
  • Systemic and cell type-specific gene expression patterns in scleroderma skin. (semanticscholar.org)
  • We found consistent differences in the patterns of gene expression between skin biopsies from individuals with scleroderma and those from normal, unaffected individuals. (semanticscholar.org)
  • Not everyone with scleroderma develops this degree of skin hardening. (blogspot.com)
  • Cosmetic surgery may help to lessen the effects of scleroderma on your skin. (familydoctor.org)
  • Localized scleroderma typically causes a skin rash that grows longer and/or deeper over time. (dukehealth.org)
  • Two trials have demonstrated a reduction of new skin ulcers for certain scleroderma patients after taking Bosentan. (umm.edu)
  • The drug was approved in Europe in 2007 for the treatment of skin ulcers related to scleroderma. (umm.edu)
  • The name scleroderma comes from the Greek words skleros , which means hard, and derma , which means skin. (umm.edu)
  • Localized scleroderma usually affects only the skin on the hands and face. (umm.edu)
  • People with systemic sclerosis have similar skin changes to those with localised disease, but these are usually much more widespread over the body. (mydr.com.au)
  • Scleroderma may be difficult to diagnose in the early stages, as some symptoms are common to some other skin and joint diseases. (mydr.com.au)
  • It is thus not surprising that the disease was originally named "scleroderma," from the ancient Greek words "σχλερος" (tight) and "δερμα" (skin) [ 2 ]. (biomedcentral.com)
  • Early life antibiotic exposure causes intestinal dysbiosis and exacerbates skin and lung pathology in experimental systemic sclerosis. (greenmedinfo.com)
  • Systemic scleroderma sine scleroderma: A challenging disease in terms of diagnostics, because the skin exhibits no symptomatology and the viscera is solely affected. (symptoma.com)
  • While the results of this process are most easily seen in the skin ("scleroderma" is literally translated "hard skin"), many other parts of the body can become involved. (houstonrheumatology.com)
  • Scleroderma means hard skin. (almondsburysurgery.nhs.uk)
  • If you have scleroderma, you have areas of hardening of the skin. (almondsburysurgery.nhs.uk)
  • In diffuse cutaneous systemic sclerosis (dcSSc) skin changes also can involve the upper arms, thighs or trunk. (almondsburysurgery.nhs.uk)
  • The word scleroderma means hardening of the skin, although the condition is not limited to the skin. (circulationfoundation.org.uk)
  • Scleroderma comes from the Greek words for hard "sclero" and skin "dermo. (sclerodermanews.com)
  • Scleroderma (sklair-oh-DUR-muh) is a group of rare diseases that involve the hardening and tightening of the skin and connective tissues. (mayoclinic.org)
  • In some people, scleroderma affects only the skin. (mayoclinic.org)
  • Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. (mayoclinic.org)
  • The skin changes associated with limited scleroderma typically occur only in the lower arms and legs, below the elbows and knees, and sometimes affect the face and neck. (mayoclinic.org)
  • In limited scleroderma, skin changes typically affect only the lower arms and legs, including fingers and toes, and sometimes the face and neck. (mayoclinic.org)
  • Limited scleroderma can cause tiny calcium deposits (calcinosis) to develop under your skin, mainly on your elbows, knees and fingers. (mayoclinic.org)
  • Barsotti S, Stagnaro C, d'Ascanio A, Della Rossa A. One year in review 2016: systemic sclerosis. (medscape.com)
  • In addition, race and ethnic background may influence the risk of getting scleroderma and the pattern of disease manifestations. (hss.edu)
  • Because the manifestations and severity of scleroderma vary among individuals, personalized medical management is essential. (scleroderma.org)
  • Additional members of the Canadian Scleroderma Research Group are shown in Appendix A . (wiley.com)
  • We conducted a cross-sectional, multicenter study of 659 patients with SSc from the Canadian Scleroderma Research Group Registry. (wiley.com)
  • The Canadian Scleroderma Research Group (CSRG) convened an expert panel in the area of nutrition to develop new recommendations in the screening and management of nutritional disorders related to scleroderma. (umm.edu)
  • Although advances in understanding in pathophysiology and use of immunosuppressive therapy has brought significant improvement in outcome of other autoimmune diseases, scleroderma still remains as a disease with high mortality and 10 yr survival rate has improved only from 54% to 66% during last 25 years1. (clinicaltrials.gov)
  • The study identifies the presence of microchimerism in male and female patients with systemic scleroderma and provides support for the hypothesis that persistent microchimerism plays a role in the pathogenesis of the disease. (springer.com)
  • Role of B cells in the pathogenesis of systemic sclerosis. (semanticscholar.org)
  • The pathogenesis of systemic sclerosis. (medscape.com)
  • Gilbane AJ, Denton CP, Holmes AM. Scleroderma pathogenesis: a pivotal role for fibroblasts as effector cells. (medscape.com)
  • Previous reports indicated the significance of the autocrine TGF-β loop in the pathogenesis of scleroderma. (jci.org)
  • in addition, some individuals have SSc overlap syndromes, which involve features of different systemic autoimmune rheumatic diseases ( SARDs ) as well as those of SSc. (arupconsult.com)
  • Overlap syndrome - May involve features of scleroderma and features of other autoimmune syndromes. (uvahealth.com)
  • citation needed] In patients with neuromuscular disorders, particularly progressive systemic sclerosis and visceral myopathy, the duodenum is frequently involved. (wikipedia.org)
  • Scleroderma is a broad term used to denote a subset of sclerosing disorders. (oncologynurseadvisor.com)
  • Watch this recording of a presentation by Philip Goglas and Dane Christiansen of Health and Medicine Counsel of Washington (HMCW), who advise the Scleroderma Foundation on important matters of public policy and legislation that affect people with scleroderma and related disorders. (scleroderma.org)
  • It may also be referred to as Progressive Systemic Sclerosis (PSSc), or Familial Progressive Systemic Sclerosis (FPSSc). (sclero.org)
  • and Familial Progressive Systemic Sclerosis, a rare form which runs in families. (sclero.org)
  • Progressive systemic sclerosis (PSS or scleroderma) occurs in men engaged in many kinds of work, and until recently there has been little reason to suspect that this disease might be preferentially associated with any particular occupation. (annals.org)
  • Myocardial lesions of progressive systemic sclerosis. (semanticscholar.org)
  • The cardiac conduction system in progressive systemic sclerosis. (semanticscholar.org)
  • An historical account of the study of progressive systemic sclerosis (diffuse scleroderma). (semanticscholar.org)
  • Electrocardiograms of ninety patients with acrosclerosis and progressive diffuse sclerosis (scleroderma). (semanticscholar.org)
  • Limited scleroderma is a progressive disorder. (umm.edu)
  • Pathology of progressive systemic sclerosis (generalized scleroderma) with special reference to changes in the viscera. (medscape.com)
  • Systemic scleroderma (also called progressive systemic sclerosis) is marked by generalized sclerotic changes in the connective tissues and finer vessels. (thefreedictionary.com)
  • Systemic sclerosis patients exhibited lower vitamin D levels compared with healthy controls. (greenmedinfo.com)
  • It is important to identify and treat the organs affected with scleroderma. (nationaljewish.org)
  • Systemic Sclerosis and Scleroderma is a serious and sometimes fatal autoimmune disease that affects a variety of organs. (biospace.com)
  • Systemic scleroderma is a condition that affects a multitude of organs and can therefore lead to the onset of various symptoms. (symptoma.com)
  • Physical examination revealed scleroderma in both arms and legs, with puffy fingers, Raynaud phenomenon and ankyloglossia. (bmj.com)
  • Preliminary criteria for the classification of systemic sclerosis (scleroderma). (nih.gov)

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