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Prevalence and Longterm Course of Primary Biliary Cirrhosis in Primary Sjögren's Syndrome GREGORIOS S. HATZIS, GEORGIOS E. FRAGOULIS, AGGELOS KARATZAFERIS, IOANNA DELLADETSIMA, CALIPSO BARBATIS, and HARALAMPOS M. MOUTSOPOULOS
ABSTRACT. 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. (First Release Aug 15 2008; J Rheumatol 2008;35:2012-6) Key Indexing Terms:
SJÖGREN'S SYNDROME
From the Departments of Pathophysiology and Pathology, National University of Athens Medical School; and Department of Pathology, Red Cross Hospital, Athens, Greece. G.S. Hatzis, MD; G.E. Fragoulis, MD; A. Karatzaferis, MD, Department of Pathophysiology; I. Delladetsima, MD, Department of Pathology, National University of Athens Medical School; C. Barbatis, MD, Department of Pathology, Red Cross Hospital; H.M. Moutsopoulos, MD, FACP, FRCP, Department of Pathophysiology, National University of Athens Medical School. Address reprint requests to Dr. H.M. Moutsopoulos, National University of Athens Medical School, Department of Pathophysiology, 75 Mikras Asias, Athens 11527, Greece. E-mail: hmoutsop@med.uoa.gr Accepted for publication May 26, 2008. |
