Autoimmune hepatitis overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Autoimmune hepatitis (AH) was first described in the 1950s and has had a variety of names including chronic active hepatitis, lupoid hepatitis, plasma cell hepatitis and autoimmune chronic active hepatitis. It is characterized by circulating autoantibodies and a high serum globulin level. It is important to distinguish AH from other forms of liver disease, as a large percentage of patients respond to therapy and therapy has been shown to delay transplantation, improve the quality of life as well as prolong survival. Lupoid hepatitis (also called Autoimmune hepatits) is an auto-immune disease which causes liver cirrhosis. It may be associated with systemic lupus erythematosus (SLE) or other connective tissue disorders. 60% of patients have chronic hepatitis that may mimic viral hepatitis, but without serologic evidence of a viral infection. The disease usually affects women and is strongly associated with anti-smooth muscle auto-antibodies. Autoimmune hepatitis is a condition in which the patient's own immune systems attacks the liver causing inflammation and liver cell death. The condition is chronic and progressive. Although the disease is chronic, many patients with autoimmune hepatitis present acutely ill with jaundice, fever and sometimes symptoms of severe hepatic dysfunction, a picture that resembles acute hepatitis.

Autoimmune hepatitis usually occurs in women (70 %) between the ages of 15 and 40. Although the term "lupoid" hepatitis was originally used to describe this disease, patients with systemic lupus erythematosus do not have an increased incidence of autoimmune hepatitis and the two diseases are distint entities. Patients usually present with evidence of moderate to severe hepatitis with elevated serum ALT and AST activities in the setting of normal to marginally elevated alkaline phosphatase and gamma-glutamyltranspeptidase activities. The patient will sometimes present with jaundice, fever and right upper quadrant pain and occasionally systemic symptoms such as arthralgias, myalgias, polyserositits and thrombocytopenia. Some patients will present with mild liver dysfunction and have only laboratory abnormalities as their initial presentation. Others will present with severe hepatic dysfunction.