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자료유형
학술저널
저자정보
저널정보
거트앤리버 발행위원회 Gut and Liver Gut and Liver 제8권 제5호
발행연도
2014.1
수록면
462 - 470 (9page)

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Recent studies have proposed nomenclatures of type 1autoimmune pancreatitis (AIP) (IgG4-related pancreatitis),IgG4-related sclerosing cholangitis (IgG4-SC), IgG4-relatedcholecystitis, and IgG4-related hepatopathy as IgG4-relateddisease (IgG4-RD) in the hepato-bilio-pancreatic system. InIgG4-related hepatopathy, a novel concept of IgG4-relatedautoimmune hepatitis (AIH) with the same histopathologicalfeatures as AIH has been proposed. Among organs involvedin IgG4-RD, associations with pancreatic and biliary lesionsare most frequently observed, supporting the novel conceptof “biliary diseases with pancreatic counterparts.” Targets oftype 1 AIP and IgG4-SC may be periductal glands around thebile and pancreatic ducts. Based on genetic backgrounds, innateand acquired immunity, Th2-dominant immune status,regulatory T (Treg) or B cells, and complement activation viaa classical pathway may be involved in the development ofIgG4-RD. Although the role of IgG4 remains unclear in IgG4-RD, IgG4-production is upregulated by interleukin 10 fromTreg cells and by B cell activating factor from monocytes/basophilswith stimulation of toll-like receptors/nucleotide-bindingoligomerization domain-like receptors. Based on thesefindings, we have proposed a hypothesis for the developmentof IgG4-RD in the hepato-bilio-pancreatic system. Furtherstudies are necessary to clarify the pathogenic mechanismof IgG4-RD.

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