title: [Treatment of systemic sclerosis].
abstract: Systemic sclerosis (SSc, scleroderma) is a multisystem disease with a bad prognosis. No drugs have been shown to cure this disease. In view of its pathogenesis, in which immune responses are presumed to play an important role, immune suppressive therapies are widely applied in the treatment of SSc. Recent placebo-controlled trials with cyclophosphamide in patients with SSc complicated by alveolitis, show a modest but significant difference in pulmonary function tests in favour for the cyclophosphamide treated group. Treatment with prostacyclin analogues and endothelin-1 receptor antagonists appear to stabilize pulmonary arterial hypertension, a thus far fatal complication of SSc.
authors.affiliation: Department of Rheumatology, Radboud University Medical Centre Nijmegen & Rheumatology Centre St Maartenskliniek, PO Box 9101, 6500 HB , Nijmegen, Niederlande. F.vandenHoogen@reuma.umcn.nl
authors.forename: F H J
authors.initials: FH
authors.lastname: van den Hoogen
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use; D004305:Dose-Response Relationship, Drug; D065128:Endothelin Receptor Antagonists; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008727:Methotrexate / Q000627:therapeutic use; D010396:Penicillamine / Q000627:therapeutic use; D016032:Randomized Controlled Trials as Topic; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D065128:Endothelin Receptor Antagonists; D007166:Immunosuppressive Agents; D014665:Vasodilator Agents; D003520:Cyclophosphamide; D010396:Penicillamine; D008727:Methotrexate