Primary biliary cirrhosis (PBC) is a chronic non-suppurative destructive cholangitis of unknown etiology. It has been reported that patients with PBC suffer from xerostomia and keratoconjunctivitis at high prevalence and PBC has been shown to be associated with Sjögren's syndrome. This study investigated the relationship between PBC and Sjögren's syndrome.
20 cases diagnosed as PBC consented to the evaluation for Sjögren's syndrome which included clinical and serological features, Schirmer's test, measurement of parotid flow rate and labial minor salivary gland biopsy.
The main manifestations of PBC are jaundice, pruritus, hepatosplenomegaly. M2 subtype of antimitochondrial antibody is of great specificity for PBC. Many PBC patients are suffering from xerostomia and keratoconjunctivitis sicca but do not have typical serological and pathological presentations of primary Sjögren's syndrome. Corticosteroids and immunosuppressive agents have disappointing effects in the treatment. Ursodeoxycholic acid is effective to certain extent.
It was indicated that Sjögren's syndrome associated with PBC appears to be a secondary form differing from primary Sjögren's syndrome.