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April 30, 1973

Section 9.—Progressive Systemic Sclerosis (Scleroderma)

JAMA. 1973;224(Suppl_5):711-716. doi:10.1001/jama.1973.03220190051012

Progressive systemic sclerosis (PSS) is a generalized disorder of connective tissue characterized by inflammatory, fibrotic, and degenerative changes, accompanied by vascular lesions, in the skin (scleroderma), synovium, and certain internal organs, notably the esophagus, intestinal tract, heart, lung, and kidney. The disease may exist in a mild form that is compatible with long life, but in many cases there is steadily and at times rapidly progressive visceral involvement leading to cardiac failure, fulminant hypertensive renal disease, pulmonary complications, or intestinal malabsorption and cachexia.1-4

The origin of PSS is obscure. The demonstration of serologic abnormalities in a high proportion of cases, together with certain morphologic and other findings, has given rise to suspicion that aberrant immunity may play an important role in pathogenesis.4

Clinical Features and Pathology.—  Progressive systemic sclerosis has been described in people of all races and appears to be global in distribution. Women are affected approximately