摘要
Introduction: We describe a case of IgG4-related sclerosing cholangitis (IgG4-SC). IgG4-SC is the biliary manifestation of a recently defined clinicopathological entity, named IgG4-related disease (IgG4-RD).
Case presentation: A 52-year-old woman with history of retroperitoneal fibrosis was admitted to our hospital for further evaluation of new-onset jaundice. Laboratory investigations revealed a rise of cholestasis indexes. Magnetic resonance (MR) examination revealed intra-hepatic bile duct dilatation and biliary stricture at the hepatic hilum, without pancreatic involvement. Corticosteroid treatment lead to an improvement of patients clinical condition and decrease of cholestasis indexes.
Conclusion: The differential diagnosis between IgG4-SC, primary sclerosing. cholangitis (PSC) and cholangiocarcinoma (CCA) is very important because of the different therapeutic approach. The diagnosis of IgG4-related sclerosing disease should be considered in patients with sclerosing cholangitis, especially when it is associated with other fibroinflammatory disorder, to avoid unnecessary surgery.
- 出版日期2015