New-onset jaundice in a patient with history of retroperitoneal fibrosis, A case report

Introduction: We describe a case of IgG4-related sclerosing cholangitis (IgG4-SC). IgG4-SC is the biliary manifestation of arecently 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 evaluationof new-onset jaundice. Laboratory investigations revealed a rise of cholestasis indexes. Magnetic resonance (MR) examinationrevealed intra-hepatic bile duct dilatation and biliary stricture at the hepatic hilum, without pancreatic involvement. Corticosteroidtreatment lead to an improvement of patient’s 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 consideredin patients with sclerosing cholangitis, especially when it is associated with other fibroinflammatory disorder, to avoid unnecessarysurgery.