Part 2 book “Sherlock’s diseases of the liver and biliary system” has contents: Autoimmune hepatitis and overlap syndromes, enterically transmitted viral hepatitis, drug - induced liver injury, iron overload states, wilson disease, nutrition and chronic liver disease, the liver in the neonate, in infancy, and childhood, and other contents. | Chapter 19 Autoimmune Hepatitis and Overlap Syndromes Ashnila Janmohamed and Gideon M. Hirschfield Centre for Liver Research, NIHR Biomedical Research Centre, University of Birmingham, Birmingham, UK LEARNING POINTS Autoimmune hepatitis (AIH) is an immune mediated liver disease that can present in all ages and races and both sexes. Disease presentation is variable, ranging from asymptomatic disease to fulminant liver failure. There is an absence of a specific diagnostic marker, hence diagnosis is made by exclusion of alternative liver disease and precipitants, for example, drugs and viruses. Clinically, AIH is characterized by raised serum alanine aminotransferase, hypergammaglobulinaemia, autoantibodies, and interface hepatitis. Seropositivity for autoantibody subclassifies disease into two distinct entities: antinuclear and/or smooth muscle antibodies (type 1 disease) and anti liver kidney microsomal 1 and/or liver cytosol 1 (type 2 disease). The cornerstone of therapy is immunosuppression using corticosteroids and azathioprine with the majority of patients achieving remission, reflecting that AIH is normally treatment responsive. Liver transplantation is indicated in patients who present with fulminant liver failure or have end stage liver disease. Lack of response to immunosuppression should prompt confirmation of compliance, exclusion of alternative/additional aetiology such as primary biliary or primary sclerosing cholangitis, and initiation of second line 967 therapy if appropriate. Lack of standardization of second line therapy in AIH demonstrates an ongoing need for new and more rational therapies. Introduction Autoimmune hepatitis (AIH) is a heterogeneous immune mediated liver disease that, in most cases, has effective treatment when diagnosed promptly and treated judiciously with classical immunosuppression focused on corticosteroids and azathioprine. The spectrum of presentation alongside the absence of clear pathophysiology and non specific .
