Cholestatic Liver Disease: Primary Biliary Cirrhosis, Primary Sclerosing Cholangitis, and Drug-Induced Cholestasis Keith D. Lindor, M.D

Autoimmune Cholangitis Autoimmune cholangitis, or AMA-negative primary biliary cirrhosis, is characterized by clinical and histologic features identical to those of primary biliary cirrhosis. However, the

Table 1. Differential Diagnosis for Cholestasis in Adults Without Biliary Obstruction

Drug-induced cholestasis Primary biliary cirrhosis

Autoimmune cholangitis Primary sclerosing cholangitis

Small-duct primary sclerosing cholangitis Idiopathic adulthood ductopenia Idiopathic biliary ductopenia Cholestasis of pregnancy Cystic fibrosis HIV-associated cholestasis Sarcoidosis Granulomatous hepatitis

anti-smooth muscle antibodies. Occasionally, these patients are confused with those who have overlapping autoimmune hepatitis and primary biliary cirrhosis, but the histologic features and biochemical profile generally help differentiate autoimmune cholangitis from the overlap between primary biliary cirrhosis and autoimmune hepatitis.