Sj?gren's syndrome in patients with primary biliary cirrhosis

Symptomatology and objective findings of Sj?gren's syndrome were evaluated in 38 consecutive patients with primary biliary cirrhosis. Symptoms of Sj?gren's syndrome were present in 18 (47.4%) patients, but were severe enough to warrant therapy in only four (10.5%). Nineteen patients consented to evaluation for Sj?gren's syndrome, which included Schirmer's I test, measurement of parotid flow rate and serum autoantibodies, labial minor salivary gland biopsy and human leukocyte antigen typing. Histological changes diagnostic of Sj?gren's syndrome were present in five patients (26.3%). All five patients had symptoms of Sj?gren's syndrome and three had abnormal Schirmer's I tests, but none had corneal ulcerations or decreased parotid flow rates. Results of serological tests and human leukocyte antigen typing were not similar to those described in patients with primary Sj?gren's syndrome but were similar to those described in patients with rheumatoid arthritis and Sj?gren's syndrome. These findings indicate that Sj?gren's syndrome associated with primary biliary cirrhosis is a form of secondary Sj?gren's syndrome resembling that associated with rheumatoid arthritis.