Autoimmune liver diseases: internist’s guide from bench to bedside

Abstract

Autoimmune liver diseases are disorders of unknown etiology and immune pathogenesis, characterized by liver parenchyma inflammation (autoimmune hepatitis) or by lesions of the intralobular biliary ducts (primary biliary cirrhosis) or of the entire biliary system (primary sclerosing cholangitis). They differ with regard to the epidemiological, clinical, morphological and serological features; the possible evolution; the different associations with other immune diseases of the digestive or extra-digestive organs; the treatment options. All progressively can result in hepatic cirrhosis. More recently, overlap syndromes have been identified, in which patients exhibit overlapping clinical, morphological and serological features of the above indicated diseases. The frequency of overlap syndromes is progressively increasing, causing additional clinical difficulties. Here, I review the diagnostic and clinical problems of the definite autoimmune liver diseases and of the overlap syndromes, with more regard to the evidences that drive current practice.

Keywords

• autoimmune liver diseases, autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, overlap syndromes.