The CREST syndrome is a variant of progressive systemic sclerosis (PSS), characterized by calcinosis (C), Raynaud's phenomenon (R), esophageal dysfunction (E), sclerodactyly (5), and telangiectasia (T). A case of incomplete CREST syndrome associated with Sjögren's syndrome is reported.
The patient was a 60-year-old Japanese woman suffering from dry mouth for 3 years and dry eyes for a year. Her initial diagnosis was sicca syndrome, but laboratory data and Raynaud's phenomenon without symptoms indicated a connective tissue disorder. Immunological data demonstrated positive values for rheumatoid factor (372U/ml) and antinuclear antibody (1: 640, centromere type). The 99mTc scintigram showed reduced uptake in both the parotid and submandibular glands. Histological examination of a lip biopsy specimen revealed perivascular and periductal lymphocytic infiltration in the labial salivary glands. For further evaluation, she was referred to the department of internal medicine. The diagnosis was incomplete CREST syndrome (CRES) with intramuscular calcification of the deltoid portion, Raynaud's phenomenon, esophageal dysfunction, and sclerodactyly.
