Prevalence and longterm course of primary biliary cirrhosis in primary Sjögren's syndrome.

Objective: To study the prevalence of primary biliary cirrhosis (PBC) and its progression in patients with primary Sjögren's syndrome (SS).

Methods: We investigated 410 patients with primary SS, without history of liver disease, for the presence of PBC based on a retrospective review of clinical, biochemical, immunologic, and histologic data.

Results: Thirty-six (8.8%) patients had cholestatic liver biochemistry. Of them, 21 (5.1%) had positive antimitochondrial autoantibodies (AMA) detected by indirect immunofluorescence, while 15 were AMA-negative. Ten of the 21 AMA-positive patients and 7 of the 15 AMA-negative patients were further investigated with liver biopsy, the result of which was compatible with PBC in all but one (AMA-negative) patient. Overall, 27 (6.6%) patients had definite (n=10), probable (n=11), or AMA-negative (n=6) PBC. Pathologically, most PBC lesions were stage 1. Five patients had a second liver biopsy, with no significant histological deterioration.

Conclusion: PBC is a rather uncommon development in patients with primary SS. The disease appears to be pathologically mild, with a propensity for slow progression, as assessed clinically, biochemically, and histologically.