Abstract
Autoimmune hepatitis (AIH) is a rare liver disease, of unknown origin, characterized by considerable heterogeneity. AIH can affect both sexes, of all ages, ethnicities and races. The revised Clinical Practice Guidelines (CPGs) of the Hellenic Association for the Study of the Liver aim to provide updated guidance to clinicians. The diagnosis of AIH is based on clinicopathological characteristics, such as elevation of immunoglobulin G (IgG) levels, detection of autoantibodies, portal or lobular hepatitis at the histological level, absence of viral hepatitis markers, and a favorable response to immunosuppressive treatment. Clinical manifestations at onset vary, from no symptoms to the fulminant form of the disease. Aminotransferases and bilirubin levels also vary, while liver biopsy is a prerequisite to establish a firm diagnosis. Investigation for detection of autoantibodies is the cornerstone for diagnosis, if it is performed according to the CPGs. Treatment of AIH should aim towards the achievement of complete biochemical response (CBR; normalization of aminotransferases and IgG) no later than 6-12 months after treatment initiation, and also histological remission of the disease. All patients with active disease, irrespective of the presence of cirrhosis, should receive personalized and response-guided first-line induction treatment with predniso(lo)ne combined with mycophenolate mofetil or azathioprine. Treatment should be given for at least 3-5 years, and for at least 2 years after the achievement of CBR, while liver biopsy should be considered before treatment cessation. The updated CPGs also provide guidance for the management of difficult-to-treat patients, including those with variants and specific forms of AIH.