Sjögren's syndrome in patients with the CREST variant of progressive systemic scleroderma.

Twenty-three patients with the CREST (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) variant of progressive systemic sclerosis, were clinically, histopathologically and serologically examined for the presence of Sjögren's syndrome (SS). Fourteen were found to be positive. No significant difference could be demonstrated between them and the remaining 9. Characteristics of patients with CREST were compared with those of 29 randomly chosen patients with primary SS. Parotid gland enlargement was more frequently present (p less than 0.01) in the latter than in the former. Virtually no patients with CREST with SS had antibodies to Ro(SSA)/La(SSB).