Autoimmune Hepatitis
Autoimmune hepatitis (AIH) is considered the result of a loss of tolerance against one’s own liver tissue, probably triggered by external factors in genetically predisposed patients. The diagnosis is based on a combination of clinical, laboratory, and his
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Autoimmune Hepatitis
Autoimmune hepatitis (AIH) is considered the result of a loss of tolerance against one’s own liver tissue, probably triggered by external factors in genetically predisposed patients. The diagnosis is based on a combination of clinical, laboratory, and histologic features, as well as the exclusion of other disorders, as illustrated in Fig. 9.16. The histologic manifestations of AIH vary depending on the presentation (e.g., acute hepatitis; asymptomatic, discovered during routine laboratory tests; fulminant hepatitis; chronic liver disease). AIH usually responds to immunosuppressive therapy,
and liver biopsy may be used to monitor the response to treatment and decide when to stop treatment. In some instances, AIH, primary biliary cirrhosis (PBC), and primary sclerosing cholangitis may be difficult to differentiate on clinical and laboratory grounds. The term overlap syndrome often is used in these cases. Other nonautoimmune conditions, such viral or drug-induced hepatitis or even steatohepatitis, may overlap serologically with AIH. In all these cases, liver histology may contribute to the final diagnosis.
A.W.H. Chan et al., Atlas of Liver Pathology, Atlas of Anatomic Pathology, DOI 10.1007/978-1-4614-9114-9_9, © Springer Science+Business Media New York 2014
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Autoimmune Hepatitis
Autoimmune Hepatitis
Fig. 9.1 Autoimmune hepatitis. A regenerative nodule in a cirrhotic liver is rimmed by dense septal lymphoplasmacytic infiltrate, with marked interface hepatitis; this is accompanied by prominent lobular necroinflammation. AIH commonly affects women, with a female-tomale ratio of 4:1. The usual age of presentation depends on the particular type of disease: bimodal (10–25 years and 45–70 years) for type 1, less than 15 years for type 2, and 37–43 years for type 3. Clinical manifestations vary widely and include asymptomatic deranged liver function (30%), acute hepatitic presentation (20%), fulminant liver failure, and cirrhosis. About half of patients suffer from concurrent autoimmune disorders, such as thyroiditis, or rheumatoid arthritis. Excellent clinical response to immunosuppression is characteristic in about 90% of patients. Different types of AIH depend on the autoantibody profile.
Fig. 9.2 Autoimmune hepatitis. A dense portal lymphoplasmacytic infiltrate and marked interface hepatitis are present. Portal plasma cell– rich inflammation typically is seen in AIH. Interface hepatitis, formerly referred to as piecemeal necrosis, is characterised by portal inflammatory cells eroding through the limiting plate between the portal tract and liver parenchyma, and surrounding individual and small groups of periportal hepatocytes. Significant interface hepatitis is a hallmark feature of AIH. Other characteristic features include emperipolesis and hepatocyte rosette formation.
Fig. 9.3 Autoimmune hepatitis. A plasma cell–rich interface hepatitis is evident. Portal plasma cell–rich inflammation is not a consistent feature of AIH and may be absent in up to one third of cases, parti
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