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. 2001 Oct;49(4):589–594. doi: 10.1136/gut.49.4.589

Autoimmune hepatitis and its variant syndromes

Z BEN-ARI 1, A CZAJA 1
PMCID: PMC1728469  PMID: 11559660

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Figure 1  .

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Diagnostic and treatment algorithm for variant syndromes of autoimmune hepatitis (AIH). Seropositivity for smooth muscle antibodies (SMA), antinuclear antibodies (ANA), and/or antibodies to liver/kidney microsome type 1 (anti-LKM1), abnormal serum gamma globulin (GG) or immunoglobulin G (IgG) level, interface hepatitis on histological examination, and/or an aggregate score of at least 10 by the modified system of the International Autoimmune Hepatitis Group support the diagnosis of AIH. Concurrent findings of antimitochondrial antibodies (AMA+), cholestatic clinical and/or laboratory features, and histological evidence of cholangitis indicate an overlap syndrome with primary biliary cirrhosis (AIH-PBC). Cholestatic clinical and/or laboratory features, absence of antimitochondrial antibodies (AMA−), concurrent chronic ulcerative colitis (CUC), and an abnormal (Abnl) cholangiogram indicate an overlap syndrome with primary sclerosing cholangitis (AIH-PSC). Similar clinical, laboratory, and histological findings in the absence of CUC and with a normal (Nl) cholangiogram suggest the outlier syndrome of autoimmune cholangitis (AIC). Treatment decisions are based on the predominant manifestations of the disease, as assessed mainly by serum alkaline phosphatase (Alk phos) or γ-glutamyl transpeptidase (GGT) levels, serum titres of ANA and/or SMA, and histological features.

Selected References

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