title: Scleroderma: developments from Osler to the present.
abstract: The clinical entity of progressive systemic sclerosis (PSS, or scleroderma) has remained unchanged since Osler's first description in 1892. Several related or overlap syndromes have now been recognized, which may afford some insight into etiologic events in the development of PSS. As yet, the cause of PSS remains elusive. Abnormalities of collagen synthesis, the role of cellular and humoral immunity, and the relationship of these to vascular disease and hyperreactivity represent current areas of research. Few therapeutic advances of proven efficacy have been forthcoming over this period except for the use of vigorous antihypertensive therapy or early nephrectomy, dialysis, and transplantation in the control of malignant hypertension and progressive renal failure.
mesh_terms: D000328:Adult; D000917:Antibody Formation; D000959:Antihypertensive Agents / Q000627:therapeutic use; D003094:Collagen / Q000096:biosynthesis; D005260:Female; D006801:Humans; D006973:Hypertension / Q000188:drug therapy / Q000209:etiology; D007111:Immunity, Cellular; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000209:etiology / Q000276:immunology / Q000628:therapy
chemical_list: D000959:Antihypertensive Agents; D003094:Collagen