Autoimmune hepatitis (AIH) is an inflammatory disorder of the liver, which was described for the first time in the 1950s by Jan G. Waldenström (Mieli-Vergani & Vergani, 2009). The exact cause of AIH is unknown (Czaja & Freese, 2002) and because of ambiguity in diagnosing the disease the exact incidence of AIH is difficult to quantify, but reported incidences range from one in 200,000 in the general population in the United States to twenty in 100,000 females in Spain (Mieli-Vergani & Vergani, 2009). Women have a higher incidence of the disease than men, with a ratio of 3.6:1; the disease is not particular to age or ethnicity. The diagnosis of AIH usually occurs after investigation when the patient complains of the symptoms such as loss of appetite,
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Some patients also have what is known as “overlap syndrome” when they display characteristics of either primary biliary cirrhosis or primary sclerosing cholangitis as well as AIH (Boberg, et al., 2011). Early diagnosis of the disease is also difficult, unfortunately, because the symptoms and presentation are extremely variable from case to case so a single test cannot serve all patients, which also presents complications when diagnosing AIH in the elderly (Hennes, et al., 2008). A number of factors must be evaluated before a definitive diagnose is made: liver histology, serum biochemistry, serum immunoglobulins, serum antibodies, viral markers, and other etiological factors (Manns, et al., 2010). In order to earn a definite diagnosis, versus a probable diagnosis, all standards of each of the six aforementioned categories must be met. The liver histology must present necrosis of at least moderate activity regardless of lobular hepatitis or central-portal bridging necrosis, but without biliary lesions or well-defined granulomas. Any abnormality in serum aminotransferases is adequate for definite diagnosis. The total serum globulin must be greater than 1.5 times the upper normal limit. The serum antibodies …show more content…
The new system is based on a point system (Table 1). A score ≥6 was defined as probable AIH; a score ≥7 was defined as definite AIH.
Three classifications were described by the IAIHG for categorizing liver histology: typical histology, histology compatible with AIH, and atypical histology (Hennes, et al., 2008). Liver histology that is typical for AIH must have interface hepatitis, emperipolesis, and hepatic rosette formation (Hennes, et al., 2008). Liver histology that is compatible for AIH does not have the features that describes the typical histology, but rather has the histology of chronic hepatitis with lymphocytic infiltration. Atypical histology does not fit either of the preceding criteria (Hennes, et al., 2008). Interface hepatitis that is present in the liver histology typical for AIH displays characteristic imbalances in the ratio of lymphocytes in the portal tract and parenchyma according to immunohistochemical tests (Mieli-Vergani & Vergani, 2009). T-cells with the α/β T-cell receptor are most numerous, and the majority of the T-cells are CD4 helper cells, while the rest are CD8 cytotoxic cells; natural killer cells, macrophages, and B-cells have a significant lower presence than the T-cells combined (Mieli-Vergani & Vergani,
Some patients also have what is known as “overlap syndrome” when they display characteristics of either primary biliary cirrhosis or primary sclerosing cholangitis as well as AIH (Boberg, et al., 2011). Early diagnosis of the disease is also difficult, unfortunately, because the symptoms and presentation are extremely variable from case to case so a single test cannot serve all patients, which also presents complications when diagnosing AIH in the elderly (Hennes, et al., 2008). A number of factors must be evaluated before a definitive diagnose is made: liver histology, serum biochemistry, serum immunoglobulins, serum antibodies, viral markers, and other etiological factors (Manns, et al., 2010). In order to earn a definite diagnosis, versus a probable diagnosis, all standards of each of the six aforementioned categories must be met. The liver histology must present necrosis of at least moderate activity regardless of lobular hepatitis or central-portal bridging necrosis, but without biliary lesions or well-defined granulomas. Any abnormality in serum aminotransferases is adequate for definite diagnosis. The total serum globulin must be greater than 1.5 times the upper normal limit. The serum antibodies …show more content…
The new system is based on a point system (Table 1). A score ≥6 was defined as probable AIH; a score ≥7 was defined as definite AIH.
Three classifications were described by the IAIHG for categorizing liver histology: typical histology, histology compatible with AIH, and atypical histology (Hennes, et al., 2008). Liver histology that is typical for AIH must have interface hepatitis, emperipolesis, and hepatic rosette formation (Hennes, et al., 2008). Liver histology that is compatible for AIH does not have the features that describes the typical histology, but rather has the histology of chronic hepatitis with lymphocytic infiltration. Atypical histology does not fit either of the preceding criteria (Hennes, et al., 2008). Interface hepatitis that is present in the liver histology typical for AIH displays characteristic imbalances in the ratio of lymphocytes in the portal tract and parenchyma according to immunohistochemical tests (Mieli-Vergani & Vergani, 2009). T-cells with the α/β T-cell receptor are most numerous, and the majority of the T-cells are CD4 helper cells, while the rest are CD8 cytotoxic cells; natural killer cells, macrophages, and B-cells have a significant lower presence than the T-cells combined (Mieli-Vergani & Vergani,