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Diffuse Scleroderma (Progressive Systemic Sclerosis)

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A rapid onset form of SYSTEMIC SCLERODERMA with progressive widespread SKIN thickening over the arms, the legs and the trunk, resulting in stiffness and disability.

Also Known As:

Progressive Systemic Sclerosis; Scleroderma, Diffuse; Scleroderma, Progressive; Sclerosis, Progressive Systemic; Sudden Onset Scleroderma; Progressive Scleroderma; Scleroderma, Sudden Onset; Sclerodermas, Sudden Onset; Sudden Onset Sclerodermas; Systemic Sclerosis, Progressive

Networked: 748 relevant articles (27 outcomes, 50 trials/studies)

Relationship Network

Disease Context: Research Results

Skin and Connective Tissue Diseases
- Skin Diseases: 8226
  - Systemic Scleroderma: 4265
    - Diffuse Scleroderma: 748
      - Diffuse systemic sclerosis: 169
- Connective Tissue Diseases: 2260
  - Systemic Scleroderma: 4265
    - Diffuse Scleroderma: 748
      - Diffuse systemic sclerosis: 169

Related Diseases

1.	Rheumatoid Arthritis
2.	Cutaneous T-Cell Lymphoma (Lymphoma, T Cell, Cutaneous)
3.	Hepatolenticular Degeneration (Wilson's Disease)
4.	Systemic Lupus Erythematosus (Libman-Sacks Disease)
5.	Fibrosis (Cirrhosis)

Experts

1.	Silman, A: 4 articles (01/2000 - 01/2000)
2.	Thompson, A: 4 articles (01/2000 - 01/2000)
3.	Fenlon, D: 4 articles (01/2000 - 01/2000)
4.	Wells, G: 4 articles (01/2000 - 01/2000)
5.	Shea, B: 4 articles (01/2000 - 01/2000)
6.	Furst, D: 4 articles (01/2000 - 01/2000)
7.	Pope, J: 4 articles (01/2000 - 01/2000)
8.	Connolly, M Kari: 3 articles (03/2010 - 10/2003)
9.	Whitfield, Michael L: 3 articles (03/2010 - 10/2003)
10.	Clements, P J: 3 articles (04/2004 - 06/2000)

Drugs and Biologics

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

1.	Penicillamine (Cuprimine)FDA Link 05/01/2008 - "To undertake a single-centre retrospective randomly selected cohort study to examine the effects of D-penicillamine treatment on skin and visceral organ involvement in patients with rapidly progressive systemic sclerosis of recent onset. " 10/01/1991 - "A 15-year (1973-1988) prospective study was conducted to determine the effectiveness of D-penicillamine (D-pen) in the treatment of rapidly progressive systemic sclerosis (SSc) of recent onset. " 10/01/1991 - "A 15-year prospective study of treatment of rapidly progressive systemic sclerosis with D-penicillamine [see comment]." 10/01/1990 - "A 58-year-old woman with progressive systemic sclerosis developed a butterfly erythematous eruption, hemorrhagic maculae, crusting, and erosions on the face 15 days following administration of D-penicillamine. " 10/01/1990 - "Lupus erythematosus-like eruption due to D-penicillamine in progressive systemic sclerosis."
2.	Methoxsalen (Oxsoralen)FDA Link 09/01/1998 - "The pharmacokinetic profile of 8-MOP has been pursued as part of a clinical study which should assess the efficacy of ECP in patients with progressive systemic sclerosis and cutaneous T-cell lymphoma. " 01/01/1998 - "The pharmacokinetic profile of 8-MOP has been pursued in a clinical study which aimed to assess the efficacy of ECP in patients with progressive systemic sclerosis and cutaneous T-cell lymphoma. "
3.	RelaxinIBA 02/01/1998 - "Longer term controlled trials are warranted to define the potential efficacy of relaxin in patients with diffuse scleroderma." 06/06/2000 - "Twenty-four weeks of recombinant human relaxin, 25 microg/kg per day, is associated with reduced skin thickening, improved mobility, and improved function in patients with moderate to severe diffuse scleroderma." 02/01/1998 - "Thirty patients with stable diffuse scleroderma of moderate severity received recombinant human relaxin at 6, 12, 50, 100, and 200 microg/kg/day or placebo in a double blind, sequential panel, dose escalation study. " 10/01/2005 - "The relaxin gene knockout mouse: a model of progressive scleroderma." 03/01/1999 - "Relaxin was recently tested in a clinical trial for efficacy in the treatment of progressive systemic sclerosis, and was administered at levels up to 10 times higher than that measured during pregnancy. "
4.	ametantrone (HAQ)IBA 04/01/2004 - "The health assessment questionnaire (HAQ) is strongly predictive of good outcome in early diffuse scleroderma: results from an analysis of two randomized controlled trials in early diffuse scleroderma." 04/01/2004 - "A low baseline HAQ (defined as less than the median HAQ score) is predictive of improved outcome in diffuse scleroderma at 1 and 2 yr." 03/01/2001 - "Improvement in the HAQ DI score in these patients with diffuse scleroderma was associated with improvement in skin thickening, hand function, oral aperture, lung function, signs of arthritis, serum creatinine level, and the investigator's global assessment of improvement. "
5.	AntibodiesIBA 10/01/1986 - "Positive results for Scl 70 antibodies were obtained in 77% of cases of diffuse scleroderma and 44% of acrosclerosis. " 09/01/2005 - "Forward-looking clinical studies that can clarify the usefulness of quantitative determination of anti-Scl-70 antibodies in the monitoring of diffuse scleroderma patients can be performed only when standard serum with a known antibody concentration and calibration curves for quantitative ELISA measurements are made available." 10/01/2012 - "Clinically two distinct subsets may be separated, acral (also termed limited) and diffuse scleroderma, which are characterized by anti-centromere and anti-Scl-70/topoisomerase-1 antibodies, respectively. " 01/01/2008 - "Kaplan-Meier univariate analysis showed that renal, cardiac involvement, pigmentation disturbances, malabsorption, a forced vital capacity <50%, diffuse scleroderma, presence of early malignancy, anaemia, and increased erythrocyte sedimentation rate (ESR) were signs of unfavourable prognosis, whereas anti-centromere antibodies were indicators of a good survival. " 03/01/2007 - "Anti-PHET antibodies were detected in 8.4% of sera of SSc patients by immunoblotting, and associated with diffuse scleroderma and lung involvement. "
6.	Interferon-gamma (Interferon, gamma)IBA 01/01/1993 - "There are very few drugs effective in the treatment of diffuse scleroderma and therefore we conducted an open study with gamma interferon. " 06/01/1991 - "In an open study conducted over 6 months, gamma interferon was administered to 12 patients suffering from progressive systemic sclerosis. " 06/01/1991 - "[Therapy of progressive scleroderma with gamma-interferon]." 08/01/1993 - "Application of interferon-gamma containing gel for local treatment of skin ulcers in autoimmune vasculitides and skin changes in progressive systemic sclerosis." 01/01/1988 - "Peripheral blood lymphocytes from patients with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), progressive systemic sclerosis (PSS), Reiter's disease, osteoarthritis, and from healthy volunteers were investigated for interferon-gamma (IFN-gamma) production after mitogen activation. "
7.	Cisapride (Propulsid)FDA Link 01/01/1989 - "Progressive systemic sclerosis Cisapride was effective in improving symptoms over a four week period. " 06/19/1990 - "We therefore compared, by means of esophageal manometry, the effect of 20 mg cisapride orally vs. placebo in 12 patients with progressive systemic sclerosis and proven esophageal dysfunction as well as in 10 healthy volunteers in a double-blind, prospective trial. " 08/01/2002 - "Effects of cisapride on colonic transit in patients with progressive systemic sclerosis." 02/01/2002 - "Effects of cisapride on oesophageal transit of solids in patients with progressive systemic sclerosis." 08/01/1987 - "The effects of cisapride on gastric emptying, esophageal emptying, and gastrointestinal symptoms were evaluated in 8 patients with progressive systemic sclerosis who had delayed gastric emptying of the solid or liquid component of a meal, or both. "
8.	Nifedipine (Adalat)FDA LinkGeneric 05/29/1986 - "The global perfusion score increased by at least 2.0 in 10 patients and decreased by at least 2.0 in only 1. These observations reveal short-term improvement in thallium-201 myocardial perfusion with nifedipine in patients with progressive systemic sclerosis. " 08/01/1986 - "Nifedipine treatment for progressive systemic sclerosis." 05/29/1986 - "Nifedipine and thallium-201 myocardial perfusion in progressive systemic sclerosis." 05/01/1985 - "Thus, nifedipine may have detrimental effects on progressive systemic sclerosis patients." 05/01/1985 - "Nifedipine and esophageal dysfunction in progressive systemic sclerosis. "
9.	CreatinineIBA 04/01/2005 - "Only 12 (2%) of the 675 patients with diffuse scleroderma had no explanation for the elevated creatinine level. " 04/01/2005 - "Patients with diffuse scleroderma without renal crisis rarely have significant increases in serum creatinine or proteinuria that cannot be explained by other etiologies. " 03/01/2001 - "Improvement in the HAQ DI score in these patients with diffuse scleroderma was associated with improvement in skin thickening, hand function, oral aperture, lung function, signs of arthritis, serum creatinine level, and the investigator's global assessment of improvement. " 09/01/1966 - "[Comparative creatine-creatinine studies in the scope of oral creatine loading in dermatomyositis and progressive scleroderma]." 08/01/1984 - "The orally active angiotensin-converting enzyme inhibitor captopril was administered for up to 11 weeks to three patients with progressive systemic sclerosis presenting with hypertension and plasma creatinine levels of 3.1, 7.2 and 10.4 mg/100 ml. Only one patient had malignant phase hypertension. "
10.	Diltiazem (Cardizem)FDA LinkGeneric 01/01/1985 - "The efficacy of diltiazem in the treatment of Raynaud's phenomenon was assessed in a prospective double-blind randomised cross-over trial in 16 patients (7 progressive systemic sclerosis, 2 rheumatoid arthritis, 1 systemic lupus erythematosus, and 6 idiopathic Raynaud's phenomenon). " 08/01/1986 - "Effects of diltiazem versus nifedipine on lower esophageal sphincter pressure in patients with progressive systemic sclerosis."

Therapies and Procedures

1.	Plasma Exchange 09/01/1995 - "[A prospective study of plasma exchange in the treatment of diffuse scleroderma]." 02/01/1990 - "Treatment of progressive systemic sclerosis by plasma exchange: long-term results in 40 patients." 01/01/1987 - "Plasma exchange in the treatment of nonadvanced stages of progressive systemic sclerosis." 11/01/1983 - "Seven patients, 4 women and 3 men afflicted with severe progressive systemic sclerosis (PSS) were treated with Plasma Exchange after failure of different other treatment. " 11/01/1983 - "Treatment of progressive systemic sclerosis with plasma exchange. "
2.	Photopheresis 06/01/1993 - "The results suggest that photopheresis may be beneficial in selected early cases of progressive systemic sclerosis." 12/01/2003 - "The benefit of extracorporeal photopheresis (ExP) in progressive systemic sclerosis (PSS) is controversial. " 12/01/2003 - "[Extracorporeal photopheresis in progressive systemic sclerosis: discrimination of responders and non-responders]." 09/01/1999 - "[Progressive systemic sclerosis - treatment results of extracorporeal photopheresis]." 05/01/1997 - "We evaluated the use of photopheresis in the treatment of patients with progressive systemic sclerosis (PSS; scleroderma). "
3.	Transplantation (Transplant Recipients) 08/01/2011 - "Treatment of severe progressive systemic sclerosis with transplantation of mesenchymal stromal cells from allogeneic related donors: report of five cases." 05/01/2008 - "Marked improvement of severe progressive systemic sclerosis after transplantation of mesenchymal stem cells from an allogeneic haploidentical-related donor mediated by ligation of CD137L." 06/01/2003 - "As such, pneumatosis intestinalis is only a sign and must be interpreted in light of the clinical findings because it may be found in various scenarios: in patients who are otherwise healthy, and associated with pyloric stenosis, jejunoileal bypass, progressive systemic sclerosis, transplantation, chemotherapy, immunosuppression (including AIDS), obstructive pulmonary disease and finally, as in our case, after liver transplantation. " 10/01/1995 - "PI has been found in several distinctive clinical settings: 1) in premature infants with necrotizing enterocolitis; 2) in adults with obstructive pulmonary disease; 3) in adults and children with a wide variety of associated conditions, including pyloric stenosis, jejunoileal bypass, progressive systemic sclerosis, transplantation, ischemic bowel, and drug therapy, particularly steroids, chemotherapy, and immunosuppression; 4) in adults as a primary benign problem; and 5) as an incidental finding in endoscopic mucosal biopsies. "
4.	Ligation 05/01/2008 - "Marked improvement of severe progressive systemic sclerosis after transplantation of mesenchymal stem cells from an allogeneic haploidentical-related donor mediated by ligation of CD137L."
5.	Drug Therapy (Chemotherapy) 01/01/1988 - "In a two-year prospective therapeutic trial, 15 patients with progressive systemic sclerosis (PSS) were treated with immunosuppressive drug therapy with or without long-term plasmapheresis. " 09/01/2013 - "Patients with a progressive systemic sclerosis and further risk factors should be treated with high-dose chemotherapy with blood stem cell transplantation before organ function is severely compromised. " 07/01/2005 - "Fatal progressive systemic sclerosis following autologous stem cell transplantation and high-dose chemotherapy." 01/01/1988 - "Visceral improvement following combined plasmapheresis and immunosuppressive drug therapy in progressive systemic sclerosis." 01/01/1987 - "Ten patients suffering from progressive systemic sclerosis, who responded poorly to conventional therapy, underwent plasma exchange (PE) therapy in conjunction with drug therapy. "