Progressive systemic sclerosis subgroups: variable pulmonary features

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Abstract
Evidence has accumulated which enables subdivision of progressive systemic sclerosis (scleroderma) into three categories: (1) classic progressive systemic sclerosis (PSS); (2) the CRST/CREST syndrome; or (3) overlap syndromes. Previous reports of the chest film manifestations of PSS have used only the broad category of scleroderma without subdivision. This study analyzed the chest film manifestations of 73 PSS patients which have been subgrouped. Radiographic abnormalities, either interstitial lung disease or pleural effusion, were present as follows: PSS, 18% (8/44); CRST/CREST, 0% (0/9); and PSS-overlap, 75% (15/20). Interstitial lung diseases in the PSS-overlap group was usually basilar and of mild severity on the initial chest film obtained. While the classic PSS group had a very much lower incidence of lung disease, it was usually more diffuse and severe.