Autoimmune liver diseases

Abstract

Autoimmune liver diseases include autoimmune hepatitis (AIH), primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Sometimes they occur simultaneously in the form of the ‘overlap syndrome’. While AIH primarily affects liver cells, the other two diseases affect biliary epithelium and typically present with pruritus, fatigue, jaundice, and cholestatic profile in laboratory findings. AIH and PBC affect predominantly females and have specific, almost pathognomonic serologic features, whereas PSC typically occurs in young men with inflammatory bowel disease. These diseases are mostly asymptomatic, or manifest themselves in nonspecific symptoms, and are therefore often detected in advanced stages of fibrosis or cirrhosis. While liver biopsy is the gold standard in the diagnosis of AIH and the overlap syndrome, MRCP is the method of choice for PSC. Regarding therapy, AIH generally responds well to immunosuppressive therapy, but requires continued treatment until biochemical and histological remission is achieved, which inevitably implies at least three years of treatment. Ursodeoxycholic acid, obeticholic acid, and ERCP in extrahepatic bile duct strictures are currently the only modalities for the treatment of cholestatic liver diseases. In decompensated liver cirrhosis or hepatocellular cancer, liver transplantation remains the last treatment option.