Clinical aspects of systemic and localized scleroderma.
Continued efforts are being made to better define the clinical course, disease subsets, and predictors of outcome in scleroderma. Data suggest that the course of the skin disease is triphasic, with the most active thickening phase in the first 12 months of disease. The presence of specific autoantibodies may predict clinical course more precisely than any clinical feature. Antipolymerase I, II, and III antibodies seem specific for scleroderma and, if present, may predict aggressive disease. Early detection of lung involvement provides an opportunity to select patients who may be responsive to drug treatment. Renal crisis in scleroderma is still important and may occur in the absence of significant signs of cutaneous fibrosis. Renin plasma levels do not appear helpful in predicting renal crisis. Significant gastrointestinal reflux disease with delayed acid clearance and esophagitis is associated with aperistalsis of the lower esophagus. Evidence for widespread structural and functional abnormalities of the microvascular circulation have been reemphasized. The psychosocial impact of scleroderma has been studied, demonstrating both the importance of depression and the need for social support. The etiology of localized scleroderma remains unknown despite efforts to link these lesions to Borrelia burgdorferi infection.
Wigley
F M
FM
Department of Medicine, Johns Hopkins University, Baltimore, MD 21205.
D001323:Autoantibodies / Q000096:biosynthesis; D005260:Female; D006801:Humans; D008297:Male; D009369:Neoplasms / Q000150:complications; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000150:complications* / Q000276:immunology
D001323:Autoantibodies