Sarcoidosis and primary biliary cirrhosis. Literature review and illustrative case

Abstract

An illustrative case of a middle-aged woman with primary biliary cirrhosis and the onset of liver disease coincident with acute, self-limited sarcoidosis is presented. The distinctive characteristics of sarcoidosis and primary biliary cirrhosis are described, and the literature regarding a number of potential relationships between these two disorders is summarized. First, the simultaneous occurrence of primary biliary cirrhosis and sarcoidosis has been documented in a few patients. Second, some of these patients had an overlap syndrome characterized by features of both disorders, inviting speculation regarding a common pathogenesis. Third, in patients with sarcoidosis, advanced cholestatic sarcoid liver disease may develop, which may cause diagnostic confusion with primary biliary cirrhosis. Finally, patients with primary biliary cirrhosis may have pulmonary fibrosis as an associated disease.