Approach to the liver biopsy diagnosis of adult cholangiopathies

Abstract

Cholangiopathy refers to a wide spectrum of chronic biliary disorders. Primary biliary cholangitis and primary sclerosing cholangitis account for the vast majority of cholangiopathies in adult patients. Inflammatory variants of these disorders are often described as overlap syndromes with autoimmune hepatitis. Other cholangiopathies show clinical and histologic features that can mimic primary biliary cholangitis and primary sclerosing cholangitis, and require clinical information as well as a careful assessment of histologic findings to arrive at the correct diagnosis. In this review, the histologic features common to chronic biliary disorders is reviewed. Following that is a discussion of primary biliary cholangitis, primary sclerosing cholangitis, and the overlap syndromes. The key entities in the differential diagnosis are described, with an emphasis on histologic and clinical features that distinguish these entities from the primary adult cholangiopathies.