Korean J Pancreas Biliary Tract.  2020 Jul;25(2):100-106. 10.15279/kpba.2020.25.2.100.

Advances in IgG4-related Hepatobiliary Disease

  • 1Department of Internal Medicine, Hallym University Sacred Heart Hospital, Anyang, Korea


The emergence of the new disease entity of steroid responsive immunoglobulin G4 (IgG4)-related hepatobiliary disease has generated considerable interest among hepatobiliary society. IgG4-related hepatobiliary disease refers to mainly IgG4-related sclerosing cholangitis (IgG4-SC), and includes a small number of IgG4-related hepatopathy. As IgG4-SC responds well to glucocorticoid therapy, IgG4-SC should be differentiated from cholangiocarcinoma and primary sclerosing cholangitis. Timely diagnosis of IgG4-SC can lead clinicians to prescribe adequate glucocorticoid treatment that can reverse bile duct strictures and cholestatic liver function. Differentiation of IgG4-SC from primary sclerosing cholangitis is sometimes challenging because serum IgG4 and tissue IgG4 have demonstrated low positive predictive value in this setting. Recent research suggested that blood IgG4/IgG RNA ratio by quantitative polymerase chain reaction can be used for differentiation. Although most patients with IgG4-SC/autoimmune pancreatitis respond to glucocorticoid therapy, they frequently experience relapse of disease. The suggested relapse factors included very high serum IgG4, diffuse enlargement of the pancreas, proximal IgG4-SC, and multi-organ involvement. This review discusses the recent advances in the pathogenesis, diagnosis, management, disease relapse, and monitoring disease activity of IgG4-SC.


자가면역췌장염; IgG4 연관 간담도 질환; IgG4 연관 경화성 담관염; IgG4 연관 질환; Autoimmune pancreatitis; IgG4-related hepatobiliary disease; IgG4-related sclerosing cholangitis; Immunoglobulin G4-related disease
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