Systemic scleroderma

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In medicine, systemic scleroderma, also called systemic sclerosis, is "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."[1]

Treatment

Clinical practice guidelines are available for treatment.[2]

Treatment options include:[3]

  • Immunosuppression
  • Autologous stem-cell transplantation
  • Treat fibrosis directly

Complications

Renal crisis

Recent increase in corticosteroid use is a risk factor for subsequent renal crisis according to a case-control[4] and cohort study.[5]

With treatment using angiotensin-converting enzyme inhibitors, an studies find that about 40% of patients die or require permanent renal dialysis.[6][7]

References

  1. Anonymous (2015), Systemic scleroderma (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. Kowal-Bielecka O, Landewé R, Avouac J, Chwiesko S, Miniati I, Czirjak L et al. (2009). "EULAR recommendations for the treatment of systemic sclerosis: a report from the EULAR Scleroderma Trials and Research group (EUSTAR).". Ann Rheum Dis 68 (5): 620-8. DOI:10.1136/ard.2008.096677. PMID 19147617. Research Blogging.
  3. Charles C, Clements P, Furst DE (2006). "Systemic sclerosis: hypothesis-driven treatment strategies.". Lancet 367 (9523): 1683-91. DOI:10.1016/S0140-6736(06)68737-0. PMID 16714190. Research Blogging.
  4. Steen VD, Medsger TA (1998). "Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis.". Arthritis Rheum 41 (9): 1613-9. DOI:<1613::AID-ART11>3.0.CO;2-O 10.1002/1529-0131(199809)41:9<1613::AID-ART11>3.0.CO;2-O. PMID 9751093. <1613::AID-ART11>3.0.CO;2-O Research Blogging.
  5. DeMarco PJ, Weisman MH, Seibold JR, Furst DE, Wong WK, Hurwitz EL et al. (2002). "Predictors and outcomes of scleroderma renal crisis: the high-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis trial.". Arthritis Rheum 46 (11): 2983-9. DOI:10.1002/art.10589. PMID 12428241. Research Blogging.
  6. Steen VD, Medsger TA (2000). "Long-term outcomes of scleroderma renal crisis.". Ann Intern Med 133 (8): 600-3. PMID 11033587[e]
  7. Walker JG, Ahern MJ, Smith MD, Coleman M, Pile K, Rischmueller M et al. (2003). "Scleroderma renal crisis: poor outcome despite aggressive antihypertensive treatment.". Intern Med J 33 (5-6): 216-20. PMID 12752889[e]