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. ...