Systemic sclerosis.
Systemic sclerosis affects the connective tissue of various organs. It is characterized by alteration of the microvasculature with a dense inflammatory cellular infiltrate, followed by massive deposition of collagen. This review discusses diagnostic criteria, clinical subsets, survival data, clinical evaluation, epidemiology, pathogenesis, immunobiology, genetics, and therapy for systemic sclerosis in adults. Because the diagnosis of systemic sclerosis is most dependent on its cutaneous involvement, knowledge of this is essential in the evaluation and management of patients with this disorder.
Perez
M I
MI
Department of Dermatology, Yale University School of Medicine, New Haven, CT 06510.
Kohn
S R
SR
D006801:Humans; D012595:Scleroderma, Systemic / Q000188:drug therapy / Q000209:etiology / Q000276:immunology / Q000473:pathology