Histology of autoimmune cholestatic liver diseases

摘要

The category of autoimmune cholestatic liver diseases comprises the entities known as primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and overlap syndromes between each of these with autoimmune hepatitis1'2. The latter (overlap) group is described in Chapter 14 of this volume.PBC and PSC are 'vanishing bile duct' diseases, characterized by (apparently at least partially) autoimmune-based destruction and disappearance of intrahep-atic bile ducts, resulting in chronic impairment of bile flow or cholestasis. It follows that the histological features in liver biopsies of patients with PBC or PSC comprise changes due to chronic cholestasis in general, besides portal alterations and bile duct lesions characteristic for the individual diseases. These are described below under the headings: liver changes of chronic cholestasis; portal and bile duct lesions in PBC; portal and bile duct lesions in PSC.