Inflammatory disease of the bile ducts-cholangiopathies: liver biopsy challenge and clinicopathological correlation

Liver biopsy interpretation in inflammatory diseases of the bile ducts or chronic cholangiopathies may be challenging, especially for pathologists working outside referral centres, where there is a limited exposure to relatively uncommon conditions. In view of the importance of sampling errors resulting from the patchy distribution of pathognomonic bile duct injuries and the misleading absence of cholestasis in the early stages, there is a need to recognize surrogate markers and subtle changes, in particular the early periportal deposition of copper and mild biliary interface activity. Such findings may either constitute the first indication of a primarily biliary disorder or be supportive of a clinically suspected diagnosis. Histological changes common to chronic cholangiopathies are reviewed at the variable stages of development that patients may first present to clinicians. As awareness of the protean clinical manifestations is essential for histological interpretation, the major and distinctive anatomoclinical features of primary biliary cirrhosis and primary and acquired sclerosing cholangitis are revisited, together with so-called overlapping syndromes and less common variants and associations, including more recently documented conditions, such as IgG4-related disease and the rarer multidrug resistance 3 deficiency. The review stresses the importance of evaluating histological changes in conjunction with clinical information.