《Table 1 Autoimmune hepatitis and IgG4-associated autoimmune hepatitis》

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《Autoimmune hepatitis and IgG4-related disease》

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c AIH:Classical autoimmune hepatitis；HPF:High-powered field；NA:Not available；IAIHG:International autoimmune hepatitis group；PSL:Prednisolone.

The first case of IgG4-AIH was reported by Umemura et al[17]in 2007.Serum levels of ALT，ALP，γGTP，IgG，and IgG4 were markedly elevated in this case.Interface hepatitis，lobular hepatitis，and rosette formation were seen in the liver biopsy specimens.This case met the diagnostic criteria for AIH proposed by the International Autoimmune Hepatitis Group（IAIHG）[18]and was diagnosed as AIH.On the other hand，this case was atypical with regard to classical AIH in that abundant infiltration of IgG4-expressing plasma cells in the portal tract and a marked elevation of serum IgG4 concentrations were observed.Based on enhanced IgG4 antibody responses，the authors proposed a new disease entity called IgG4-AIH[17].Following this case report，five groups，including ours，tried to identify and characterize IgG4-AIH in patients who met the diagnostic criteria for AIH（Table 1）[7-11].