Systemic Scleroderma (Systemic Sclerosis)

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.

Also Known As:

Networked: 4265 relevant articles (178 outcomes, 495 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Interstitial Lung Diseases (Interstitial Lung Disease)
2.	Ulcer
3.	Fibrosis (Cirrhosis)
4.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
5.	Sclerosis

Experts

1.	Sato, Shinichi: 80 articles (12/2015 - 06/2002)
2.	Distler, Oliver: 58 articles (01/2016 - 01/2002)
3.	Hasegawa, Minoru: 57 articles (09/2015 - 06/2002)
4.	Allanore, Yannick: 55 articles (10/2015 - 01/2003)
5.	Matucci-Cerinic, Marco: 53 articles (07/2015 - 01/2002)
6.	Denton, Christopher P: 50 articles (07/2015 - 05/2003)
7.	Distler, Jörg H W: 46 articles (01/2016 - 02/2004)
8.	Takehara, Kazuhiko: 46 articles (09/2015 - 06/2002)
9.	Mayes, Maureen D: 44 articles (12/2015 - 09/2003)
10.	Fujimoto, Manabu: 44 articles (09/2015 - 06/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Systemic Scleroderma:

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 12/31/2007 - "Clinically modest short-term efficacy of cyclophosphamide treatment has been shown in systemic sclerosis, but was not maintained at 2 years." 04/01/2002 - "To evaluate, in a pilot study, the efficacy of a short term cyclophosphamide (CYC) pulse regimen on alveolitis in a cohort of patients with systemic sclerosis (SSc). " 11/01/2006 - "This study aims to analyze the effects of cyclophosphamide pulse therapy on parameters of lung function in patients with systemic sclerosis. " 01/01/2006 - "Low-dose intravenous cyclophosphamide in systemic sclerosis: an open prospective efficacy study in patients with early diffuse disease." 06/01/2005 - "The ongoing European ASTIS trial will compare the respective benefits of these 2 cyclophosphamide regimens in severe Systemic sclerosis."
2.	Idiopathic pulmonary hypertensionIBA 01/01/2013 - "This is a rare case of rapidly progressive pulmonary arterial hypertension associated with systemic sclerosis that can be markedly improved with early diagnosis and treatment." 04/01/2009 - "Pulmonary arterial hypertension in systemic sclerosis: can we predict responders for successful therapy?" 04/01/2012 - "Pulmonary arterial hypertension therapy may be safe and effective in patients with systemic sclerosis and borderline pulmonary artery pressure." 06/01/2007 - "Pulmonary arterial hypertension (PAH) has poor prognosis in systemic sclerosis (SSc), and at present the "gold standard" for diagnosis and follow-up of PAH in SSc is right heart catheterization (RHC) but it would be very useful to have a noninvasive way to follow these patients. " 10/01/2013 - "Survival in systemic sclerosis with pulmonary arterial hypertension has not improved in the modern era."
3.	bosentan (Tracleer)FDA Link 04/01/2015 - "We present a challenging case of PAH in a patient with systemic sclerosis in whom a marked decrease in functional capacity after the initiation of bosentan therapy led to the diagnosis of PVOD. " 10/01/2011 - "Bosentan is effective against digital ulcerations and hyperkeratosis in systemic sclerosis." 07/01/2015 - "Bosentan blocks the antiangiogenic effects of sera from systemic sclerosis patients: an in vitro study." 12/01/2006 - "Bosentan increases myocardial perfusion and function in systemic sclerosis: a magnetic resonance imaging and Tissue-Doppler echography study." 07/01/2015 - "In systemic sclerosis (SSc), clinical evidence has shown that Bosentan may foster the regeneration of the peripheral microcirculatory network. "
4.	Iloprost (Ventavis)FDA Link 05/01/2006 - "Hepatic perfusion significantly improved after Iloprost administration, suggesting that such treatment might be useful in preventing vascular complications in patients with systemic sclerosis. " 09/01/1998 - "The results on the daily duration of Raynaud's attacks suggest that both 50 and 100 microg oral iloprost twice daily may be effective in the treatment of Raynaud's phenomenon secondary to systemic sclerosis. " 01/01/2012 - "The aim of the present study was to retrospectively evaluate response to therapy in 73 patients affected by systemic sclerosis (SSc) who underwent long-term cyclic treatment with intravenous iloprost for peripheral vascular involvement (average duration of treatment 54.12±41.04 months). " 09/01/2008 - "Low versus high-dose iloprost therapy over 21 days in patients with secondary Raynaud's phenomenon and systemic sclerosis: a randomized, open, single-center study." 02/01/2008 - "The aim of this study is to evaluate the effect of iloprost on oxidative status in ten patients with systemic sclerosis by measuring urinary levels of 8-isoprostaglandin-F(2alpha), a member of F(2)-isoprostanes. "
5.	Endothelin Receptors (Endothelin Receptor)IBA 06/01/2009 - "Improved endothelial function after endothelin receptor blockade in patients with systemic sclerosis." 05/01/2015 - "It is generally accepted that blockade of endothelin receptors has potentially beneficial effects on vasculopathy associated with systemic sclerosis (SSc). " 01/01/2013 - "Longterm effects of endothelin receptor antagonism on microvascular damage evaluated by nailfold capillaroscopic analysis in systemic sclerosis." 01/01/2012 - "Endothelin receptor antagonists: effects on extracellular matrix synthesis in primary cultures of skin fibroblasts from systemic sclerosis patients." 02/01/2011 - "Endothelin receptor antagonists as disease modifiers in systemic sclerosis."
6.	imatinib (Gleevec)FDA Link 02/01/2011 - "Whether imatinib will be tolerable or effective in the treatment of systemic sclerosis is the subject of several investigations. " 01/01/2013 - "Ethnic difference may contribute to the efficacy of low-dose imatinib mesylate in systemic sclerosis." 02/01/2012 - "Efficacy of low-dose imatinib mesylate for cutaneous involvement in systemic sclerosis: a preliminary report of three cases." 02/01/2012 - "Findings from ongoing studies of imatinib in systemic sclerosis (SSc) were eagerly awaited in 2011, but results from these clinical trials have so far been disappointing. " 09/01/2012 - "Low-dose imatinib in the treatment of severe systemic sclerosis: a case series of six Chinese patients and literature review."
7.	Cyclosporine (Ciclosporin)FDA LinkGeneric 07/01/1991 - "In this open pilot study, the potential therapeutic efficacy of Cyclosporin A (CsA) in systemic sclerosis (scleroderma) was investigated. " 02/01/1994 - "Improvement in esophageal motor abnormalities in systemic sclerosis patients treated with cyclosporine: comment on the article by Clements et al." 07/01/1991 - "Efficacy of cyclosporin A in systemic sclerosis." 10/01/2001 - "The aims of the present study were: 1) to verify the tolerability of long-term, low-dose treatment of patients affected by systemic sclerosis with cyclosporin A; 2) to analyze the clinical outcome of treated patients in relationship to skin, esophageal, lung, kidney and microvascular organ involvement. " 01/01/1990 - "Cyclosporin in localized and systemic scleroderma--a clinical study."
8.	AntibodiesIBA 03/01/2013 - "Clinical significance of coexisting antitopoisomerase I and anticentromere antibodies in patients with systemic sclerosis: a EUSTAR group-based study." 01/01/2012 - "We undertook this study to determine the clinical and serologic associations of anti-Ro52/TRIM21 antibodies in patients with systemic sclerosis (SSc). " 10/01/2010 - "This study sets the ground for future investigations on a possible relationship between antibody specificity and clinical manifestations of systemic sclerosis, as well as on a possible role of FOXE3 in priming these antibodies." 05/01/2010 - "In the present study we analyzed the association of peripheral vascular events and anticardiolipin (aCl) antibodies in 54 patients suffering from systemic scleroderma. " 01/01/2005 - "The aim of our study was to evaluate the prevalence of anticardiolipin and anti-beta2-glikoprotein I (anti-beta2GPI) antibodies in patients with systemic sclerosis (SSc) and to correlate the presence of these antibodies with clinical and serological features of the disease. "
9.	Penicillamine (Cuprimine)FDA Link 08/01/1985 - "These data suggest that D-penicillamine may be useful in the treatment of systemic sclerosis that involves the lung; however, further studies are needed." 06/01/2013 - "From a dermatologic standpoint, penicillamine was found to be useful in the treatment of systemic sclerosis. " 12/01/1999 - "[D-penicillamine: an outdated cure for systemic scleroderma?]." 06/01/2001 - "To compare the systemic sclerosis (SSc) patients entered into the d-penicillamine trial with SSc patients entered into previous controlled SSc trials. " 03/01/2001 - "The Disability Index of the Health Assessment Questionnaire is a predictor and correlate of outcome in the high-dose versus low-dose penicillamine in systemic sclerosis trial."
10.	Collagen Type I (Type I Collagen)IBA 04/01/2006 - "The pleiotropic protective effects of statins on various endothelial and immune cell functions in conjunction with their potent inhibitory effects on type I collagen gene expression suggest that statins may be effective therapeutic agents in systemic sclerosis." 12/01/1994 - "The results of this study suggest that peripheral blood mononuclear cells from patients with systemic sclerosis are specifically sensitized to human type I collagen to produce increased levels of IL-6, which may play a role in the pathogenesis in this fibrotic disorder." 01/01/2013 - "Th17 cells favor inflammatory responses while inhibiting type I collagen deposition by dermal fibroblasts: differential effects in healthy and systemic sclerosis fibroblasts." 08/01/2004 - "Dosage effects of orally administered bovine type I collagen on immune function in patients with systemic sclerosis." 03/01/2004 - "Scleroderma or SSc (systemic sclerosis) is a fibrotic disease characterized by excessive production of extracellular matrix components, especially type I collagen. "

Therapies and Procedures

1.	Photopheresis 03/01/1992 - "In a pilot study of extracorporeal photochemotherapy, two patients with systemic sclerosis who received this therapy experienced significant clinical improvement. " 05/01/2006 - "Our aim was to evaluate the efficacy of photopheresis in the treatment of patients with systemic sclerosis (scleroderma). " 02/01/2012 - "The aim of this study was to evaluate the clinical and immunomodulatory effects of extracorporeal photochemotherapy (ECP) in systemic sclerosis (SSc). " 05/01/2006 - "A randomized, double-blind, placebo-controlled trial of photopheresis in systemic sclerosis." 01/01/1998 - "Thus far, the action of extracorporeal photopheresis (ECP) in severe systemic scleroderma has been evaluated in short-term studies, and only limited experience has been obtained with long-term application. "
2.	Transplants (Transplant) 09/01/2009 - "The leading hypothesis, suggesting that semi-allogenic fetal T cells may trigger a graft-versus-host type of disease, has been supported by several studies showing an increased frequency of fetal-cell microchimerism (FMc) in women affected with systemic sclerosis. " 07/01/2015 - "The authors' approach of using fat graft in the face and adipose-tissue-derived stromal vascular fraction for hands is presented as innovative and promising therapy for patients with systemic sclerosis. " 09/01/2012 - "Because scleroderma-like skin lesions can occur in chronic GVHD (cGVHD) in allogeneic stem-cell transplant (HCT) patients and hCMV is relevant in the pathogenesis of systemic sclerosis (SSc), we evaluated the possible pathogenetic link between hCMV and skin cGVHD. " 03/15/2011 - "To describe renal shielding techniques and dosimetry in delivering total body irradiation (TBI) to patients with severe systemic sclerosis (SSc) enrolled in a hematopoietic stem cell transplant protocol. " 07/01/2009 - "Autologous hematopoietic stem cell transplant in systemic sclerosis: quantitative high resolution computed tomography of the chest scoring."
3.	Stem Cell Transplantation 11/01/2013 - "Retrospective analyses of independent cohorts of systemic sclerosis patients treated with autologous stem cell transplantation showed significant improvement of skin thickening, lung function and quality of life, but at the expense of 6-17% treatment-related mortality. " 02/01/2012 - "Autologous stem cell transplantation (aSCT) for systemic sclerosis (SSc) has been shown to be effective in recent reports. " 11/01/2013 - "The purpose of this review is to discuss recent published clinical and mechanistic studies on stem cell transplantation for systemic sclerosis and their implications for clinical practice. " 05/01/2013 - "Update on stem cell transplantation for systemic sclerosis: recent trial results." 08/06/2011 - "Non-randomised studies of haemopoietic stem-cell transplantation (HSCT) in systemic sclerosis have shown improvements in lung function and skin flexibility but high treatment-related mortality. "
4.	Phototherapy (Light Therapy) 03/12/2003 - "Ultraviolet A1 (340-400 nm, UVA1) phototherapy is highly effective in sclerotic lesions of systemic sclerosis (SSc). " 06/01/2009 - "This study would suggest that UVA1 phototherapy is effective for scleroderma affecting proximal and acral sites in patients with systemic sclerosis." 10/01/2000 - "These studies indicate that UVA1 phototherapy is effective for patients with systemic sclerosis." 10/01/2000 - "Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis. " 06/01/2009 - "Previous studies report the benefit of UVA1 phototherapy in treating acrosclerosis in patients with systemic sclerosis. "
5.	Lasers (Laser) 07/01/2008 - "The method was tested against a standard laser Doppler imaging system, which to date is considered the gold standard for non-invasive assessment of cutaneous blood flow, on both healthy subjects and patients suffering from systemic sclerosis (SSc; a pathological condition with microvessel endothelium injury). " 01/01/2014 - "The objectives of this study were to evaluate the dynamic behaviour of digital skin microvascular blood flow before and after cold stimulation using laser Doppler imaging (LDI) in children and adolescents with RP secondary to juvenile systemic sclerosis (JSS), primary RP (PRP) and healthy controls and to compare functional abnormalities measured by LDI with structural microvascular abnormalities evaluated by nailfold capillaroscopy (NFC). " 06/01/2010 - "Peripheral blood perfusion correlates with microvascular abnormalities in systemic sclerosis: a laser-Doppler and nailfold videocapillaroscopy study." 03/01/2010 - "The objective of this study was to investigate the dynamic behavior of the blood flow of the microvascular circulation of the fingertips before and after two cold stimuli (CS), using Laser Doppler Imaging with different intensities in patients with systemic sclerosis (SSc) and in healthy individuals. " 08/06/2002 - "To investigate the skin of patients with systemic sclerosis using confocal laser scanning microscopy in vivo and to develop histometric parameters to describe characteristic cutaneous changes of systemic sclerosis observed by this new technique, we conducted an exploratory study. "