[Systemic sclerosis].
Systemic sclerosis (SSc; syn. systemic scleroderma) is a rare autoimmune disorder with characteristic cutaneous manifestations. Prevalence in women is fivefold higher than in men. The course of the disease is slowly progressive with a variable degree of internal organ involvement due to fibrosis and obliteration of small vessels. The diffuse form shows more frequent and severe organ manifestations compared to the limited form. Increased mortality is particularly related to a cardiopulmonary involvement leading to a 5-year survival of around 75%. Treatment indications are dependent on the severity of the disease. In the acute state, immunosuppressive agents are needed in case of significant organ involvement. Vasodilative drugs are often used for the symptomatic treatment of Raynaud's phenomenon.
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Schwerpunkt Rheumatologie und Klinische Immunologie, Medizinische Klinik und Poliklinik II, Universität Würzburg, Klinikstrasse 6-8, 97070, Würzburg, Germany. Kleinert_S@klinik.uni-wuerzburg.de
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D001323:Autoantibodies / Q000097:blood; D002503:Centromere / Q000276:immunology; D003430:Cross-Sectional Studies; D004264:DNA Topoisomerases, Type I / Q000276:immunology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D011928:Raynaud Disease / Q000175:diagnosis / Q000188:drug therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000188:drug therapy / Q000401:mortality; D012737:Sex Factors; D016019:Survival Analysis; D014665:Vasodilator Agents / Q000627:therapeutic use
D001323:Autoantibodies; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D004264:DNA Topoisomerases, Type I