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Extrahepatic manifestations of autoimmune hepatitis

INTRODUCTION

Autoimmune hepatitis (AIH) is a chronic hepatitis of unknown etiology characterized by immunologic and autoimmunologic features . (See "Clinical manifestations and diagnosis of autoimmune hepatitis".) 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.

EXTRAHEPATIC DISORDERS

Several extrahepatic disorders associated with AIH have been described (table 1). 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 to 22 percent of 63 patients with chronic viral hepatitis [1]. 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 antiendomysial antibodies to detect celiac disease or routine proctosigmoidoscopy for the detection of ulcerative colitis).

Extrahepatic disorders are seen in both type 1 and type 2 AIH (table 2) (see "Classification of autoimmune hepatitis" [2]. Associated diseases are seen in both types of AIH [3,4].

Antiendomysial antibodies, anti-gluten antibodies and anti-tissue transglutaminase antibodies reflect the co-existence of celiac disease in AIH and other autoimmune liver diseases [5-7]. The prevalence of celiac disease in AIH may be as high as 10 percent. Because liver transglutaminase levels may be elevated in patients with liver disease, it has been hypothesized that circulating anti-tissue transglutaminase levels may merely reflect liver damage; more likely they reflect the co-existence of celiac sprue and AIH.

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Last literature review version 18.2: May 2010
This topic last updated: September 25, 2009
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