자연경과에서 비대상성 간경변으로 진행한 IgG4 연관 경화성 담관염 = IgG4-Related Sclerosing Cholangitis Showing Natural Course of Progression to Decompensated Liver Cirrhosis

다국어 초록 (Multilingual Abstract)

Immunoglobulin G4-related disease (IgG4-RD) is characterized by extensive IgG4-positive plasma cells infiltration resulting in inflammation and fibrosis of the affected organs. Patient with IgG4-RD shows an excellent response to glucocorticoid therapy especially when given at early onset stages. IgG4-related sclerosing cholangitis (IgG4-SC) is considered biliary manifestation of IgG4-RD. The natural history and longterm prognosis of IgG4-SC is not well defined and most of the previous case series have reported short duration of follow-up. We present the case of development of decompensated liver cirrhosis from IgG4-related sclerosing cholangitis with a 6-year natural clinical course.
Korean J Pancreas Biliary Tract 2017;22(4):179-183

참고문헌 (Reference)

1 Huggett MT, "Type 1 autoimmune pancreatitis and IgG4-related sclerosing cholangitis is associated with extrapancreatic organ failure, malignancy, and mortality in a prospective UK cohort" 109 : 1675-1683, 2014

2 Hart PA, "Treatment of relapsing autoimmune pancreatitis with immunomodulators and rituximab: the Mayo Clinic experience" 62 : 1607-1615, 2013

3 Topazian M, "Rituximab therapy for refractory biliary strictures in immunoglobulin G4-associated cholangitis" 6 : 364-366, 2008

4 Ghazale A, "Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy" 134 : 706-715, 2008

5 Stone JH, "IgG4-related disease" 366 : 539-551, 2012

6 Ohara H, "Clinical diagnostic criteria of IgG4-related sclerosing cholangitis 2012" 19 : 536-542, 2012

7 Nakazawa T, "A case of advanced-stage sclerosing cholangitis with autoimmune pancreatitis not responsive to steroid therapy" 11 : 58-60, 2010