Systemic Scleroderma (Systemic Sclerosis)

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2550 relevant articles (113 outcomes, 290 trials/studies) found for this Disease

Description: 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:

Systemic Sclerosis; Scleroderma, Systemic; Sclerosis, Systemic

Relationship Network

Disease Context: Research Results

Skin and Connective Tissue Diseases

Skin Diseases: 5944

Systemic Scleroderma: 2550

Diffuse Scleroderma: 711

Limited Scleroderma: 34

Connective Tissue Diseases: 1633

Systemic Scleroderma: 2550

Diffuse Scleroderma: 711

Limited Scleroderma: 34

Related Diseases

1.	Sclerosis
2.	Interstitial Lung Diseases (Interstitial Lung Disease)
3.	Pulmonary Fibrosis (Hamman Rich Syndrome)
4.	Ulcer
5.	Raynaud Disease (Raynaud's Disease)

Experts

1.	Matucci-Cerinic, Marco: 22 articles (05/2008 - 01/2002)
2.	Takehara, K: 21 articles (07/2008 - 01/2000)
3.	Sato, S: 21 articles (07/2008 - 01/2000)
4.	Hasegawa, Minoru: 17 articles (09/2008 - 04/2003)
5.	Sato, Shinichi: 16 articles (09/2008 - 04/2003)
6.	Hasegawa, M: 15 articles (07/2008 - 01/2000)
7.	Takehara, Kazuhiko: 13 articles (08/2008 - 04/2003)
8.	Matucci-Cerinic, M: 12 articles (09/2008 - 05/2001)
9.	Furst, Daniel E: 11 articles (06/2008 - 11/2002)
10.	Allanore, Yannick: 11 articles (06/2008 - 01/2003)

Drugs and Biologics

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

1.	Cyclophosphamide (Cytoxan)FDA Link 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 - "OBJECTIVE: 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." Order ALL the reference details at left...
2.	Iloprost (Ventavis)FDA Link 05/01/2006 - "CONCLUSION: 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 - "CONCLUSION: 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" 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" 09/01/2003 - "Although the effect of this treatment regimen should be proven in further long-term studies, we think that an intermittent continuous therapy with iloprost could result in an improvement or stabilization of systemic sclerosis." Order ALL the reference details at left...
3.	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." 01/01/1993 - "To evaluate safety and efficacy of cyclosporin A (CSA) treatment in systemic sclerosis (SSc)" 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" Order ALL the reference details at left...
4.	AntibodiesIBA 01/01/2005 - "PURPOSE: 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" 03/01/1997 - "OBJECTIVE: To study the prevalence and specificity of antinucleolar antibodies (ANoA) in patients with systemic sclerosis (SSc), their spouses, and their first-degree relatives, and to investigate whether SSc family members have greater frequency of ANoA than expected" 10/01/1995 - "This study investigated the prevalence and clinical significance of anti-ribosomal P protein (anti-P) antibodies in patients with systemic sclerosis (SSc)" 05/01/1994 - "Anti-centromere antibodies (ACA) in systemic sclerosis patients and their relatives: a serological and HLA study." 01/01/1988 - "Studies on anti-granulocyte and anti-platelet antibodies in patients with systemic sclerosis" Order ALL the reference details at left...
5.	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." 06/01/2008 - "OBJECTIVE: To investigate the safety and efficacy of oral bovine type I collagen (CI) treatment in patients who have had diffuse cutaneous systemic sclerosis (dcSSc; scleroderma) for <or=10 years" 06/01/2008 - "A multicenter, randomized, double-blind, placebo-controlled trial of oral type I collagen treatment in patients with diffuse cutaneous systemic sclerosis: I. oral type I collagen does not improve skin in all patients, but may improve skin in late-phase disease." 12/01/1994 - "CONCLUSIONS: 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." 08/01/2004 - "Dosage effects of orally administered bovine type I collagen on immune function in patients with systemic sclerosis." Order ALL the reference details at left...
6.	Methotrexate (Mexate)FDA LinkGeneric 01/01/2000 - "Methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis" 08/01/1998 - "BACKGROUND: Low-dose methotrexate (MTX) has been shown to be effective in the treatment of systemic sclerosis" 11/01/2008 - "Indirect evidence for the efficacy of methotrexate in diffuse systemic sclerosis." 01/01/2005 - "Placebo controlled trial of methotrexate in systemic sclerosis." 04/01/1996 - "In this study, methotrexate (MTX) was compared with placebo in the treatment of systemic sclerosis (scleroderma, SSc) in a 24 week randomized double-blind trial, followed by an observational trial of 24 weeks duration" Order ALL the reference details at left...
7.	Penicillamine (Cuprimine)FDA Link 05/01/2008 - "CONCLUSIONS: In a population of patients with diffuse cutaneous systemic sclerosis, with progressive disease of recent onset, D-penicillamine treatment at a median dose of 750 mg per day caused a statistically significant reduction in skin involvement and improvement of renal, cardiac and pulmonary involvement." 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." 12/01/1999 - "D-penicillamine: an outdated cure for systemic scleroderma?]" 10/01/2006 - "Minimally important difference in diffuse systemic sclerosis: results from the D-penicillamine study." 02/01/2004 - "High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial: lessons learned." Order ALL the reference details at left...
8.	Interferon Type II (Interferon, gamma)IBA 11/01/2002 - "Four open studies have also confirmed the efficacy of interferon gamma in systemic sclerosis" 10/01/1992 - "A phase I/II trial to examine the safety and efficacy of interferon-gamma (IFN gamma) therapy for patients with systemic sclerosis (SSc)" 04/01/1999 - "Increased interferon-gamma (IFN-gamma) levels produced in vitro by alloactivated T lymphocytes in systemic sclerosis and Raynaud's phenomenon." 10/01/1998 - "Interferon-gamma in the treatment of systemic sclerosis: a randomized controlled multicentre trial." 08/15/1998 - "Treatment with interferon gamma enhances fibrinolysis in systemic sclerosis." Order ALL the reference details at left...
9.	beraprostIBA 07/01/2002 - "This study showed that DLCO levels in patients with systemic sclerosis improved after the administration of beraprost sodium, probably due to the decrease in pulmonary vascular resistance accompanied by increased cardiac output." 10/01/1999 - "OBJECTIVE: To compare the efficacy of 6 to 12 months of beraprost sodium (BPS) therapy with placebo in the prevention of digital ulceration in patients with systemic sclerosis (SSc)" 02/01/1995 - "Efficacy of beraprost sodium on Raynaud's phenomenon in patients with systemic sclerosis]" 06/01/1996 - "We investigated the effect of beraprost sodium (BPS) on the Raynaud's phenomenon on 15 patients with systemic sclerosis, 3 with mixed connective tissue disease and 1 with Raynaud's disease, respectively" 07/01/2002 - "The objective of this study was to assess the effect of beraprost sodium, an oral prostacyclin analogue, on pulmonary function in patients with systemic sclerosis" Order ALL the reference details at left...
10.	CollagenIBA 07/01/1988 - "Aggregation to collagen at all concentrations was significantly greater (p less than 0.01) in the patients with systemic sclerosis than the normal controls, with a four-fold reduction in the ED50 for SS (0.044 +/- 0.03 mcg/1) compared with controls (0.12 +/- 0.008 mcg/1)" 10/01/2008 - "This study investigated the contents and distribution of collagen V (Col V) in skin lesions of the patients with systemic sclerosis (SSc) and its roles in the pathogenesis" 08/01/2008 - "The aim of this study was to determine the role of Akt in collagen deposition by normal dermal fibroblasts, and to determine the sensitivity of cultured systemic sclerosis (SSc) fibroblasts to Akt inhibition" 02/01/2001 - "OBJECTIVE: To study concentration changes in collagen degradation markers in patients with diffuse and limited cutaneous systemic sclerosis and patients with scleroderma-related diseases" 10/01/2000 - "OBJECTIVE: To study collagen metabolites in systemic sclerosis (SSc) and their relationship with clinical manifestations of the disease" Order ALL the reference details at left...

Therapies and Procedures

1.	Photopheresis 03/01/1992 - "BACKGROUND AND DESIGN--In a pilot study of extracorporeal photochemotherapy, two patients with systemic sclerosis who received this therapy experienced significant clinical improvement" 05/01/2006 - "OBJECTIVE: Our aim was to evaluate the efficacy of photopheresis in the treatment of patients with systemic sclerosis (scleroderma)" 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" 12/01/1994 - "Furthermore, a randomized, single-blinded trial involving 79 patients with early onset, aggressive systemic sclerosis suggested that photopheresis could benefically affect the course of the cutaneous thickening in this form of the disease" Order ALL the reference details at left...
2.	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)" 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" 07/01/2007 - "To investigate microvascular function using laser Doppler imaging (LDI) following response to hyperaemia, neurovascular reflex and iontophoresis in systemic sclerosis (SSc) in comparison with primary Raynaud's phenomenon (PRP) and age-matched controls" 08/01/2004 - "OBJECTIVE: To test the hypothesis that microvascular vasodilation is impaired in patients with systemic sclerosis (SSc) compared with patients with primary Raynaud's phenomenon (PRP) and healthy controls, using the technique of laser Doppler imaging to quantify blood flow responses to iontophoresis of vasoactive agents" 08/06/2002 - "CONCLUSIONS: Although the predominant histopathological features in systemic sclerosis are findings of the reticular dermis and the subcutis, and in histopathological investigation the epidermis seems to remain unaffected by the disease, we have demonstrate some characteristic differences in the epidermis and papillary dermis by confocal laser scanning microscopy in vivo" Order ALL the reference details at left...
3.	Phototherapy (Light Therapy) 03/12/2003 - "BACKGROUND: Ultraviolet A1 (340-400 nm, UVA1) phototherapy is highly effective in sclerotic lesions of systemic sclerosis (SSc)" 10/01/2000 - "CONCLUSION: These studies indicate that UVA1 phototherapy is effective for patients with systemic sclerosis." 10/01/2000 - "OBJECTIVE: Our purpose was to determine whether UVA1 phototherapy is effective for systemic sclerosis" 12/01/2007 - "Ultraviolet A1 phototherapy for treatment of acrosclerosis in systemic sclerosis: controlled study with half-side comparison analysis." 05/01/2004 - "Low-dose UVA1 phototherapy in systemic sclerosis: effects on acrosclerosis." Order ALL the reference details at left...
4.	Stem Cell Transplantation 10/01/2005 - "Autologous Stem cell Transplantation International Scleroderma (ASTIS) trial: hope on the horizon for patients with severe systemic sclerosis." 05/01/2002 - "In systemic sclerosis, criteria for patient selection and a limited number of stem cell transplantation regimens have been agreed on and controlled trials are underway." 02/01/2008 - "CURRENT KNOWLEDGE AND KEY POINTS: Autologous haematopoietic stem cell transplantation has become a curative option for condition with very poor prognosis as severe systemic sclerosis, lupus erythematosus or other systemic diseases" 01/01/2008 - "Long-term follow-up results after autologous haematopoietic stem cell transplantation for severe systemic sclerosis." 03/01/2005 - "Red cell fragmentation preceding a renal crisis in a patient with systemic sclerosis undergoing haematopoietic stem cell transplantation." Order ALL the reference details at left...
5.	Transplants (Transplant) 11/01/2004 - "The rationale behind autologous autoimmune hematopoietic stem cell transplant conditioning regimens: concerns over the use of total-body irradiation in systemic sclerosis." 04/01/2004 - "A modified model of graft-versus-host-induced systemic sclerosis (scleroderma) exhibits all major aspects of the human disease." 04/23/1998 - "CONCLUSIONS: Fetal antimaternal graft-versus-host reactions may be involved in the pathogenesis of systemic sclerosis in some women." 04/23/1998 - "This finding suggests that fetal cells persisting in the maternal circulation or tissues could be involved in the pathogenesis of systemic sclerosis by initiating a graft-versus-host reaction" 08/01/1990 - "The first case was a 44-year-old man with systemic sclerosis and the second a 52-year-old woman who had a renal transplant" Order ALL the reference details at left...

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