RT Journal Article SR Electronic T1 Prevalence and Longterm Course of Primary Biliary Cirrhosis in Primary Sjögren’s Syndrome JF The Journal of Rheumatology JO J Rheumatol FD The Journal of Rheumatology SP 2012 OP 2016 VO 35 IS 10 A1 GREGORIOS S. HATZIS A1 GEORGIOS E. FRAGOULIS A1 AGGELOS KARATZAFERIS A1 IOANNA DELLADETSIMA A1 CALIPSO BARBATIS A1 HARALAMPOS M. MOUTSOPOULOS YR 2008 UL http://www.jrheum.org/content/35/10/2012.abstract AB 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.