INTRODUCTION
Autoimmune hepatitis is an uncommon, inflammatory liver disease that is characterized by circulating autoantibodies and elevated serum immunoglobulin G (IgG) levels (table 1). Initial therapy usually consists of oral glucocorticoids with or without a thiopurine, and most patients achieve biochemical remission with pharmacologic treatment. However, some patients may not respond to therapy and develop progressive disease and cirrhosis.This topic will discuss management of autoimmune hepatitis. The pathogenesis, clinical features, and diagnosis of autoimmune hepatitis are discussed separately. (See "Autoimmune hepatitis: Pathogenesis" and "Overview of autoimmune hepatitis".)
The overlap of autoimmune hepatitis with other immune-mediated disorders (eg, primary biliary cholangitis) is discussed separately. (See "Autoimmune hepatitis variants: Definitions and treatment".)
PRETREATMENT EVALUATION
The goals of the pretreatment evaluation are to assess risk for adverse effects related to therapy. Prior to initiating therapy, we measure the following:●Thiopurine methyltransferase (TPMT) enzyme activity. We assess TPMT enzyme activity because thiopurines (ie, azathioprine or 6-mercaptopurine) have been associated with bone marrow suppression in patients with low or absent TPMT activity (which leads to the preferential production of 6-thioguanine) (figure 1). Testing for TPMT activity and thiopurine drug metabolism are discussed in more detail separately. (See "Thiopurines: Pretreatment testing and approach to therapeutic drug monitoring for adults with inflammatory bowel disease".)