Autoimmune hepatitis: diagnostic criteria, subclassifications, and clinical features
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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Autoimmune Hepatitis
2023, MacSween's Pathology of the Liver, Eighth EditionPathologic features and differential diagnosis of chronic hepatitis
2023, Diagnostic HistopathologyCitation Excerpt :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
Salivary immunoglobulin levels in juvenile autoimmune hepatitis
2018, Archives of Oral BiologyAutoimmune Hepatitis
2018, MacSween's Pathology of the LiverChapter 76 - Cirrhosis and portal hypertension: Pathologic aspects
2016, Blumgart's Surgery of the Liver, Biliary Tract and Pancreas: Sixth Edition