• Diffuse cutaneous systemic sclerosis (also known as systemic sclerosis or scleroderma) is a chronic autoimmune disorder that affects the skin and internal organs. (rarediseaseshealthcenter.com)
  • Phototherapy using longer-wavelength ultraviolet A (UVA) light (ie, UVA1, 340-400 nm) has proved beneficial for cutaneous lesions in scleroderma. (medscape.com)
  • 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)
  • They are also referred to as anti-DNA topoisomerase I antibody (anti-topo I). Anti Scl-70 antibodies (also called anti-topoisomerase I after the type I topoisomerase target) is a type of antinuclear autoantibody seen mainly in diffuse systemic scleroderma, but is also seen the more limited form of systemic scleroderma called CREST syndrome. (wikipedia.org)
  • HLA-DR2 (DR15 and DR16) are associated with scleroderma and systemic sclerosis. (wikipedia.org)
  • Systemic scleroderma, or sclerosis -- May affect large areas of skin and organs such as the heart, lungs, or kidneys. (medlineplus.gov)
  • Systemic sclerosis (SSc) differs from localized scleroderma in prognosis as well as clinical expression. (skintherapyletter.com)
  • Dobrota et al 1 have analysed the EUSTAR (EULAR Scleroderma Trials and Research) systemic sclerosis (SSc, scleroderma) database using a subset of diffuse cutaneous SSc (dcSSc) to determine predictors of skin improvement over 1 year in randomised trials. (bmj.com)
  • Updates in Systemic Sclerosis Treatment and Applicability to Pediatric Scleroderma. (scleroderma.org)
  • Scleroderma is an aspect of systemic sclerosis , a systemic connective tissue disease that also involves subcutaneous tissue, muscles, and internal organs. (medscape.com)
  • It is characterized by microcirculation abnormalities and scleroderma and/or visceral sclerosis. (scirp.org)
  • Systemic sclerosis is a type of scleroderma, which means hard skin. (patient.info)
  • The team tested oestradiol and scleroderma autoantibody levels in banked samples from 83 men aged 50 years and older with diffuse cutaneous systemic sclerosis, a type of scleroderma. (hospitalhealthcare.com)
  • Male patients with diffuse cutaneous scleroderma had significantly higher levels of estradiol (average, 30.6pg/ml) than both healthy men (average, 12.9pg/ml) and postmenopausal women with the disease (24.2pg/ml). (hospitalhealthcare.com)
  • Scleroderma, or systemic sclerosis, is an autoimmune connective tissue disorder characterized by excessive collagen production, leading to skin thickening and fibrosis. (alliedacademies.org)
  • A total of 19 of the 35 patients in this study had limited cutaneous systemic sclerosis, 5 had diffuse cutaneous systemic sclerosis, 3 had mixed connective tissue disease, and 2 had systemic sclerosis sine scleroderma. (sidemast.org)
  • Scleroderma, or progressive systemic sclerosis, is an autoimmune connective tissue disease that involves sclerotic changes of the skin and may involve internal organs. (logicalimages.com)
  • Systemic sclerosis sine scleroderma - Raynaud phenomenon and systemic involvement without skin sclerosis. (logicalimages.com)
  • Keloidal scleroderma is a very rare cutaneous presentation of scleroderma, in which keloidal plaques develop in normal or sclerotic skin. (logicalimages.com)
  • The systemic sclerosis overlap syndrome is characterized by features of one of the scleroderma subsets with those of another autoimmune disease (eg, lupus erythematosus , dermatomyositis , Sjögren syndrome , and/or rheumatoid arthritis ). (logicalimages.com)
  • This may be due to their likelihood of having diffuse, rather than limited, cutaneous systemic scleroderma. (logicalimages.com)
  • In systemic sclerosis sine scleroderma, patients have systemic sclerosis-related antibodies and visceral manifestations of the disease but no skin tightening. (msdmanuals.com)
  • However, diffuse cutaneous scleroderma comes on suddenly, usually starting with the skin thickening on your fingers. (privatelondonrheumatologist.com)
  • The association of the limited cutaneous form of systemic scleroderma (lcSSc) and PBC is known as Reynolds syndrome. (journalmc.org)
  • Scleroderma, also called systemic sclerosis, is an autoimmune disease that produces scars and patches of thick, rough, or scaly skin. (facty.com)
  • This form of the disease is classified as diffuse cutaneous systemic sclerosis (dcSSc). (medlineplus.gov)
  • Van de 375 patiënten met dcSSc die werden gerandomiseerd naar lenabasum, tweemaal daags 5 of 20 mg, of placebo, waren de ACR CRISS-scores na 52 weken "hoog en vergelijkbaar" in alle 3 de groepen, met mediane scores rond de 0,8 zonder significante verschillen. (mednet.nl)
  • Diffuse cutaneous systemic sclerosis (dcSSc). (patient.info)
  • Cutaneous manifestations vary, with two main subsets: limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). (alliedacademies.org)
  • The purpose of this phase 2 multicenter, randomized, double-blind, placebo-controlled, study is to assess the safety and efficacy of ifetroban in patients with diffuse cutaneous systemic SSc (dcSSc) or SSc-associated pulmonary arterial hypertension (SSc-PAH). (researcherprofiles.org)
  • Markers associated with SSc were further investigated according to whether patients had limited (lcSSc) or diffuse (dcSSc) cutaneous fibrosis. (bath.ac.uk)
  • Patients who had systemic sclerosis with an onset of the first non-Raynaud's symptom within the past 7 years and a high-resolution computed tomographic scan that showed fibrosis affecting at least 10% of the lungs were randomly assigned, in a 1:1 ratio, to receive 150 mg of nintedanib, administered orally twice daily, or placebo. (nih.gov)
  • Anabasum has generated positive data in Phase 2 studies in diffuse cutaneous systemic sclerosis and cystic fibrosis. (biospace.com)
  • The Company expects to commence a Phase 2 study in systemic lupus erythematosus, a Phase 3 study in systemic sclerosis and a Phase 2b study in cystic fibrosis in the fourth quarter of 2017. (biospace.com)
  • Systemic sclerosis (SSc) is a multisystem autoimmune disease characterized by vasculopathy and fibrosis of the skin and certain internal organs. (biomedcentral.com)
  • To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center. (jrheum.org)
  • Systemic sclerosis (SSc) is an orphan connective tissue disease characterized by progressive fibrosis and vasculopathy affecting the skin and multiple internal organs [ 1 ]. (biomedcentral.com)
  • Diffuse cutaneous systemic sclerosis - proximal extremity or trunk skin sclerosis, Raynaud phenomenon of shorter duration, high risk of renal crisis, and cardiac and lung fibrosis. (logicalimages.com)
  • To investigate whether six microsatellite markers known to map closely to genes involved in fibrosis are associated with systemic sclerosis (SSc). (bath.ac.uk)
  • These associations indicate a possible role for TGFβ3, TGFβ2 and TIMP1 in genetic susceptibility to SSc and for TGFβ3 in determining the degree of cutaneous fibrosis. (bath.ac.uk)
  • Systemic sclerosis is a rare chronic disease of unknown cause characterized by diffuse fibrosis and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower gastrointestinal tract, lungs, heart, and kidneys). (msdmanuals.com)
  • Diffuse myocardial fibrosis or cardiac conduction abnormalities occur. (msdmanuals.com)
  • In patients with diffuse cutaneous systemic sclerosis (SSc), inhibitors such as the Tripterygium wilfordii derivative celastrol have been shown to block the ways in which cells perceive matrix stiffness and thus may be beneficial in alleviating skin fibrosis. (rarediseaseadvisor.com)
  • Although the terpenoid celastrol is a YAP inhibitor, whether celastrol can alleviate SSc-related fibrosis remains to be elucidated, with the cell niches necessary for cutaneous fibrosis still unknown. (rarediseaseadvisor.com)
  • Limited cutaneous systemic sclerosis (lcSSc). (patient.info)
  • In limited cutaneous systemic sclerosis (lcSSc) this affects the face, forearms and lower legs up to the knee. (patient.info)
  • It may be classified as diffuse cutaneous SSc or limited cutaneous SSc (lcSSc) on the basis of the extent and distribution of skin involvement. (journalmc.org)
  • In patients with systemic sclerosis-related interstitial lung disease, a clinically meaningful decline of 10% or more in lung function was seen in 24.5% of placebo recipients, compared with 8.6% of tocilizumab recipients. (medscape.com)
  • Interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis. (medscape.com)
  • Echocardiography is the most frequently used modality for pulmonary artery hypertension, which is present in 10-15% of patients with systemic sclerosis. (medscape.com)
  • Musculoskeletal involvement is a major complication for patients with systemic sclerosis (SSc). (acrabstracts.org)
  • Sakata K, Kaneko Y, Yasuoka H, Yamaoka K, Takeuchi T. Association of Radiographic Findings in Hand X-Ray with Autoantibodies in Patients with Systemic Sclerosis [abstract]. (acrabstracts.org)
  • This investigation involved analyses of the potential for pulmonary hypertension (PH) and interstitial lung disease (ILD) to have an impact on patients living with systemic sclerosis. (ajmc.com)
  • For patients already living with systemic sclerosis, also having both pulmonary hypertension (SSc-PH) and interstitial lung disease (SSc-ILD) exacerbates their risk of all-cause mortality, and this risk only goes up with increasing severity of both comorbidities, reports a recent published study in Pulmonary Circulation . (ajmc.com)
  • Clinical characteristics and outcomes in pulmonary manifestations of systemic sclerosis: Contribution from pulmonary hypertension and interstitial lung disease severity. (ajmc.com)
  • Limited cutaneous systemic sclerosis - distal skin sclerosis, Raynaud phenomenon, frequent severe late-stage complications such as pulmonary hypertension and gastrointestinal involvement. (logicalimages.com)
  • We conducted a randomized, double-blind, placebo-controlled trial to investigate the efficacy and safety of nintedanib in patients with ILD associated with systemic sclerosis. (nih.gov)
  • CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias-although not all are needed for the disorder to be called CREST) is an older term used to describe this subset of limited cutaneous systemic sclerosis. (medscape.com)
  • Cutaneous lupus erythematosus (CLE) is a subset of systemic lupus erythematosus (SLE) that primarily affects the skin. (alliedacademies.org)
  • no clinical benefit of nintedanib was observed for other manifestations of systemic sclerosis. (nih.gov)
  • Systemic sclerosis is a multisystem disorder with vascular instability as a clinical hallmark. (skintherapyletter.com)
  • The purpose of this article is to integrate renal and cutaneous aspects of disease as well as highlight some important, although frequently underappreciated, clinical or laboratory findings that ally renal and skin diseases. (medscape.com)
  • Systemic sclerosis is a complex and heterogeneous disease with clinical forms ranging from limited skin involvement (limited cutaneous systemic sclerosis) to forms with diffuse skin sclerosis and severe and often progressive internal organ involvement (diffuse cutaneous systemic sclerosis), and occasionally a fulminant course (fulminant systemic sclerosis). (medscape.com)
  • Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? (rsu.lv)
  • Dive into the research topics of 'Are diffuse and limited juvenile systemic sclerosis different in clinical presentation? (rsu.lv)
  • Dermatologists can assist in the management of these patients by facilitating early diagnosis, and treating cutaneous manifestations such as Raynaud's phenomenon, cutaneous calcinosis, and digital ulceration. (skintherapyletter.com)
  • The earliest symptom of systemic sclerosis is a problem called Raynaud's phenomenon. (patient.info)
  • if you have Raynaud's phenomenon it does NOT necessarily mean that you will develop systemic sclerosis. (patient.info)
  • Raynaud's phenomenon on its own is a common condition and most people who have Raynaud's phenomenon will not develop systemic sclerosis. (patient.info)
  • The term systemic sclerosis is used to describe a systemic autoimmune disease of unknown origin characterized by excessive deposition of collagen and other connective tissue macromolecules in skin and multiple internal organs, prominent and often severe fibroproliferative alterations in the microvasculature, and numerous humoral and cellular immunologic abnormalities. (medscape.com)
  • Systemic sclerosis is an autoimmune disease. (patient.info)
  • Systemic sclerosis (SSc) is a chronic, autoimmune disease that primarily affects connective tissue. (bvsalud.org)
  • Symptoms indicative of skin activity such as severe cutaneous itching or burning. (mayo.edu)
  • What are the symptoms of Diffuse cutaneous systemic sclerosis? (rarediseaseshealthcenter.com)
  • There is no cure for diffuse cutaneous systemic sclerosis, but there are medications that can help manage the symptoms. (rarediseaseshealthcenter.com)
  • They can also cause lesser degrees of limitations in two or more organs or body systems, and when associated with symptoms or signs, such as severe fatigue, fever, malaise, diffuse musculoskeletal pain, or involuntary weight loss, can also result in extreme limitation. (ssa.gov)
  • There are many possible symptoms and problems that may develop with systemic sclerosis because the extent of the disease can vary greatly from person to person. (patient.info)
  • People with systemic sclerosis should have regular monitoring of blood pressure, blood and urine tests, and lung function, and should report any unexplained symptoms promptly to a doctor. (patient.info)
  • There are lots of possible symptoms of systemic sclerosis. (patient.info)
  • These cutaneous findings can occur in isolation or in combination with systemic symptoms, aiding in the diagnosis and management of lupus [ 1 ]. (alliedacademies.org)
  • Acute symptoms were similar to those of eosinophilic myalgia syndrome, with prominent systemic symptoms in addition to cutaneous changes. (medscape.com)
  • Individuals with diffuse systemic sclerosis may experience waxing and waning symptoms that fluctuate over months or years. (facty.com)
  • On the other hand, cutaneous systemic sclerosis typically causes worsening symptoms. (facty.com)
  • People with diffuse skin disease (rather than limited skin involvement) may be more prone to progressive and internal organ disease. (medlineplus.gov)
  • High-resolution ultrasound imaging of skin involvement may be helpful to quantitatively evaluate dermal thickness in systemic sclerosis. (medscape.com)
  • Patients have acral sclerosis associated with vascular instability in addition to internal organ involvement. (skintherapyletter.com)
  • Diffuse cutaneous systemic sclerosis refers to skin thickening affecting the trunk and the skin of the extremities proximal to the elbows and knees besides involvement of the face. (medscape.com)
  • Of those with cardiopulmonary testing, 3% of diffuse cutaneous juvenile systemic sclerosis and 23% of limited cutaneous juvenile systemic sclerosis group had cardiac involvement (p = 0.015), and 41% diffuse cutaneous juvenile systemic sclerosis and 22% of the limited cutaneous juvenile systemic sclerosis group had pulmonary involvement (p = 0.009). (rsu.lv)
  • Discussion: The majority of this international juvenile systemic sclerosis cohort had diffuse cutaneous juvenile systemic sclerosis (72.5%) with more frequent vascular and pulmonary involvement compared to the limited cutaneous group, who had increased cardiac involvement. (rsu.lv)
  • Additionally, the absences of Raynaud phenomenon, abnormal capillaroscopy findings, and visceral involvement are key findings that differentiate eosinophilic fasciitis (EF) from systemic sclerosis. (medscape.com)
  • In generalized systemic sclerosis with diffuse skin involvement, patients have Raynaud syndrome and gastrointestinal (GI) complications. (msdmanuals.com)
  • Systemic sclerosis varies in severity and progression, ranging from generalized skin thickening with rapidly progressive and often fatal visceral involvement (diffuse systemic sclerosis) to isolated skin involvement (often just the fingers and face) and slow progression (often several decades) before visceral disease develops. (msdmanuals.com)
  • Visceral involvement in eosinophilic fasciitis is generally absent, a finding that helps differentiate eosinophilic fasciitis from systemic sclerosis and other differential considerations. (medscape.com)
  • Progressive systemic sclerosis predominantly affects women (3 to 8 women vs. 1 man). (scirp.org)
  • Limited cutaneous systemic sclerosis involves areas distal to the elbows and knees but may involve the face and neck. (medscape.com)
  • Current treatment of systemic sclerosis is directed toward managing complications and providing symptomatic relief. (medscape.com)
  • The phase III RESOLVE-1 lenabasum trial was a double-blinded randomized placebo-controlled trial in patients with diffuse systemic sclerosis looking at a novel strategy, which was a cannabinoid-2 receptor agonist in the treatment of systemic sclerosis. (mednet.nl)
  • We didn't model this expecting the degree of a CRISS response in our placebo group, but the landscape has changed in terms of how much mycophenolate is now being used in the treatment of systemic sclerosis. (mednet.nl)
  • The revised criteria for the classification of systemic sclerosis are listed in Table I, below. (medscape.com)
  • CTD associated with ILD include systemic sclerosis (SSc), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), polymyositis and dermatomyositis (PM/DM), Sjögren's syndrome, and mixed connective tissue disease (MCTD) 4 . (jrheum.org)
  • The association between PBC and other autoimmune conditions, such as Sjögren's syndrome, rheumatoid arthritis, Hashimoto's thyroiditis or systemic sclerosis occurs in 84% of cases [ 1 , 2 , 6 - 10 ]. (journalmc.org)
  • A pharmacokinetic study on the subject was conducted in patients with diffuse cutaneous SSc, with the findings published in Annals of the Rheumatic Diseases . (rarediseaseadvisor.com)
  • Ambartsumyan L, Zheng HB, Iyer RS, Soares J, Henstorf G, Stevens AM. Relationship between esophageal abnormalities on fluoroscopic esophagram and pulmonary function testing in juvenile systemic sclerosis . (scleroderma.org)
  • They are seen by both rheumatologists and dermatologists, who participate in the management of cutaneous complications of the disease. (skintherapyletter.com)
  • Our cohort reflects prior findings of published juvenile systemic sclerosis cohorts and emphasizes a difference in the presentation compared to adult-onset systemic sclerosis. (rsu.lv)
  • The findings show the challenge in differentiating a primary tumor of the sweat gland from a metastatic cutaneous tumor of mammary carcinoma, even with the immunohistochemical resources currently available. (bvsalud.org)
  • A very important recent study advocates a baseline chest CT for all juvenile onset systemic sclerosis patients, which parallels adult organ screening recommendations. (scleroderma.org)
  • Systemic sclerosis (SSc) is a connective tissue disease characterized by inflammatory, fibrotic and degenerative changes. (journalmc.org)
  • Diffuse cutaneous disease (skin thickness spreading above the elbows and knees) is much more common than limited cutaneous disease in children. (scleroderma.org)
  • Patients with diffuse cutaneous SSc and other characteristics associated with SSc-ILD severity had worse PRO measure scores. (nih.gov)
  • Injury to the skin and underlying tissues from acute exposure to a large external dose of radiation is referred to as cutaneous radiation injury (CRI). (cdc.gov)
  • Intravenous prostanoid therapy is recommended for severe systemic sclerosis-related digital vasculopathy, but the evidence, until now, has been scant. (sidemast.org)
  • Active digital tip ulcerations were present in 29% diffuse cutaneous juvenile systemic sclerosis and none in the limited cutaneous juvenile systemic sclerosis subjects (p = 0.005). (rsu.lv)
  • Curr Opin In- ed of multiple cutaneous ulcerations, The annual incidence of lep- fect Dis. (cdc.gov)
  • 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against Rheumatism collaborative initiative. (medscape.com)
  • Methods: Patients fulfilling the Paediatric Rheumatology European Society juvenile systemic sclerosis classification criteria were included. (rsu.lv)
  • This study was conducted prospectively to investigate the difference in manifestations of limited cutaneous juvenile systemic sclerosis and diffuse cutaneous juvenile systemic sclerosis subtypes. (rsu.lv)
  • They performed comparative studies of the cutaneous malignancy and breast nodules, especially with immunohistochemical examinations. (bvsalud.org)
  • In addition, uremia and conditions associated with renal replacement therapy are fraught with numerous and, often, relatively unique cutaneous disorders. (medscape.com)
  • Many cutaneous disorders experienced by patients undergoing dialysis have little to do with the uremic syndrome and are related to the same underlying pathologic process that caused the renal disease. (medscape.com)
  • These systemic disorders and the associated renal diseases and cutaneous manifestations are tabulated in Table 1, below. (medscape.com)
  • The objective of this presentation is to guide any practitioner towards diagnosis of renal crisis in the face of sudden multiple organ failure resulting from progressive systemic sclerosis. (scirp.org)
  • However, the ASTIS (Autologous hematopoietic stem cell transplantation vs. intravenous pulse cyclophosphamide in diffuse cutaneous systemic sclerosis) trial for stem cell transplantation versus cyclophosphamide had no upper limit of mRSS (inclusion criteria of mRSS of at least 15) and the mean baseline mRSS was 25-26. (bmj.com)
  • Introduction: Juvenile systemic sclerosis is an orphan disease. (rsu.lv)
  • Currently, the majority of juvenile systemic sclerosis cohort studies are retrospective in design without standardized assessment. (rsu.lv)
  • An additional aim was to compare these data to other juvenile systemic sclerosis cohorts and a large adult systemic sclerosis cohort. (rsu.lv)
  • Results: In all, 88 patients with a mean disease duration of 3.5 years were enrolled, 72.5% with diffuse cutaneous juvenile systemic sclerosis with a mean modified Rodnan Skin score of 18 and 27.5% with limited cutaneous juvenile systemic sclerosis with mean modified Rodnan Skin score of 9. (rsu.lv)
  • Physician global disease damage assessment was higher in the diffuse cutaneous juvenile systemic sclerosis group compared to the limited cutaneous juvenile systemic sclerosis group: 35 and 15 (p = 0.021). (rsu.lv)
  • The student who understands the details of the disease will look another level further and know that there is a different antibody in the diffuse form of the disease and choose C. (prospectivedoctor.com)
  • For example, in cutaneous lupus erythematosus, the butterfly rash may not be present in all cases, and the distribution and appearance of skin lesions can differ. (alliedacademies.org)
  • We selected the combined response index in systemic sclerosis as our primary outcome. (mednet.nl)
  • This study aimed to report and discuss the challenging differential diagnosis between a primary tumor of sweat glands and cutaneous metastasis of mammary carcinoma using anatomopathological and imaging diagnostic resources available today. (bvsalud.org)
  • The physical examination revealed sclerodactyly, cutaneous calcifications, digital ulcers and facial telangiectasias. (journalmc.org)
  • Physical examination showed mild skin thickening of forearms and moderate skin thickening of hands (according to modified Rodnan skin score), sclerodactyly, cutaneous calcifications in the forearms, pitting digital ulcers in the 2nd and 3rd fingers of the right hand and facial telangiectasias. (journalmc.org)
  • Additionally, the Company is evaluating anabasum in open-label extension studies in systemic sclerosis and skin-predominant dermatomyositis. (biospace.com)
  • The cutaneous manifestations of dermatomyositis include a heliotrope rash, Gottron's papules, nailfold changes, and a unique skin rash known as 'shawl sign. (alliedacademies.org)
  • About Diffuse cutaneous systemic sclerosis trials. (lifeclinicaltrials.com)
  • Our results suggest mechanisms of action for HICS, including upregulation of α-MSH, that has been shown to be anti-fibrotic in preclinical models, and possible markers to be included in future trials targeting skin in diffuse cutaneous systemic sclerosis. (quanterix.com)
  • Migra- diagnosis of CL was confi rmed with disseminated cutaneous leishmaniasis tion of laborers is associated with an histopathology, culture, and PCR. (cdc.gov)