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Autoimmune hepatitis: Associated extrahepatic disorders

Michael A Heneghan, MD, MMedSc, FRCPI
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Kristen M Robson, MD, MBA, FACG


Autoimmune hepatitis is a chronic hepatitis of unknown etiology characterized by immunologic and autoimmunologic features (see "Autoimmune hepatitis: Clinical manifestations and diagnosis"). In the spectrum of autoimmune disease, classified from organ-specific to non-organ-specific, autoimmune liver diseases fall somewhere in the middle. The diseases are usually confined to the liver but can be associated with a number of other autoimmune diseases.

This topic will review the extrahepatic disorders associated with autoimmune hepatitis. The hepatic manifestations, diagnosis, and treatment of autoimmune hepatitis are discussed elsewhere. (See "Autoimmune hepatitis: Clinical manifestations and diagnosis" and "Autoimmune hepatitis: Treatment".)


Several extrahepatic disorders associated with autoimmune hepatitis (AIH) have been described (table 1) and are seen in both type 1 and type 2 AIH (table 2) [1-4]. Concurrent immunologic diseases are more common with AIH than with viral hepatitis. One prospective study, for example, found that concurrent immunologic disease was present in 38 percent of 122 patients with AIH compared with 22 percent of 63 patients with chronic viral hepatitis [5]. The nature of the extrahepatic diseases was the same in both groups.

The associated diseases may antedate the appearance of AIH or occur after the diagnosis of the liver disease is established. The clinical expression of the associated disease can be extremely variable and, at times, may even be occult and unrecognized unless specifically sought (such as measurement of antibodies to detect celiac disease or routine proctosigmoidoscopy for the detection of ulcerative colitis).

Concurrent extrahepatic autoimmune disorders associated with AIH include autoimmune thyroiditis, diabetes, rheumatoid arthritis, and ulcerative colitis (ranging from approximately 20 to 49 percent of patients with AIH in various reports from around the world) [1-4,6-12]. Up to 43 percent of patients with AIH report a family history of another autoimmune disease in their first-degree relatives, commonly thyroid disease and type 1 diabetes [13].

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Literature review current through: Dec 2017. | This topic last updated: Sep 24, 2015.
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