Elsevier

Clinics in Liver Disease

Volume 6, Issue 3, August 2002, Pages 605-621
Clinics in Liver Disease

Autoimmune hepatitis: diagnostic criteria, subclassifications, and clinical features

https://doi.org/10.1016/S1089-3261(02)00019-3Get rights and content

Section snippets

Diagnosis

Because no pathognomonic features exist for this syndrome [12], [23], diagnosis requires the presence of a combination of suggestive clinical and laboratory abnormalities together with exclusion of other possible causes of liver disease (Table 1). In particular, viral hepatitis, Wilson's disease, α1-antitrypsin deficiency, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) need to be excluded by appropriate investigations. Other etiologic factors that need to be carefully

Classification

From the preceding discussion, it is apparent that there is considerable variability between patients in mode of presentation, severity of disease, and in response to therapy (see discussion in Chapter 7). For this reason, attempts have been made over the years to classify AIH according to various parameters not only for diagnostic purposes but also for assisting in focusing investigations, for accurately defining prognosis, and for planning treatment strategies.

Summary

The diagnosis of AIH depends on the finding of several suggestive features together with careful exclusion of liver diseases of other etiologies. Wherever possible, the diagnosis should be confirmed histologically by an experienced hepatopathologist. Seronegativity for the conventional autoantibodies at presentation does not exclude a diagnosis of AIH. It is important to test for anti-LKM1 antibodies to avoid missing a diagnosis of type 2 AIH, with potentially serious consequences. Although the

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      AIH may present as acute hepatitis, chronic hepatitis, and cirrhosis. It is usually associated with elevated serum transaminase levels, hypergammaglobulinemia (IgG levels >1.5 times above normal), and seropositivity for anti-nuclear (ANA), smooth muscle (SMA), and/or liver-kidney microsomal (LKMA) antibodies.13,14 Although a diagnosis of AIH is supported by positive serologic results and/or a scoring system proposed by the International Autoimmune Hepatitis Group,13,14 it is worth noting that these serologic markers are not entirely specific for AIH and can be positive in other liver diseases, including fatty liver disease15 and with HBV or HCV infection.14,16

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