Clinical, histologic, and immunopathologic features of primary biliary cirrhosis

This review of clinical and morphologic features of PBC deals with four processes in the evolution of the disease: (1) etiologic and predisposing features; (2) injury of bile ducts and secondarily of hepatocytes; (3) precholestasis and cholestasis; and (4) hepatic derangement not of a biliary nature. It further refers to four stages in the evolution of PBC — ductal, ductular, scarring, and cirrhotic stage — with extensive transitions between each other and recurrence of previous stages in later ones. Probably a different immunologic process is responsible for the initial and continuing bile duct injury and for a secondary “hepatitic” component as well as for the periductular fibrosis. The further evolution of PBC, however, appears to be dominated by the cholestatic consequences of mechanical fibrotic interference with biliary secretion, mainly in the periportal area. Assisted by progression of the “ hepatitic” process, it results to varying degrees in destruction of the hepatic architecture, potentially terminating in cirrhosis. But PBC is not a primary cholestatic disease; frank cholestasis is clinically and histologically a relatively late phenomenon in a disease still mysterious and with a potentially long evolution. Cirrhosis, which is the conventional name of the disease, is only present or even in development during a short terminal fraction of the total life span of the disease. Although PBC is a relatively rare disease, even if now more readily recognized, it nevertheless serves as a useful experiment of nature for clarifying the pathology of liver disease in general and its immunologic aspects in particular.