Z Gastroenterol 2011; 49 - P425
DOI: 10.1055/s-0031-1285696

IgG4-related sclerosing cholangitis mimicking cholangiocarcinoma in 10 patients

C Agne 1, F Puls 2, T Weismüller 1, H Bektas 3, HH Kreipe 2, MP Manns 1, K Rifai 1
  • 1Hannover Medical School, Department of Gastroenterology, Hepatology and Endocrinology, Hannover, Germany
  • 2Hannover Medical School, Department of Histopathology, Hannover, Germany
  • 3Hannover Medical School, Department of General, Visceral and Transplant Surgery, Hannover, Germany

Background & aims: IgG4 related sclerosing cholangitis (IgG4-SC) is a recently recognized form of sclerosing cholangitis. It is frequently associated with IgG-4-related autoimmune pancreatitis (AIP). It may represent a manifestation of a multisystem fibroinflammatory disorder. Lymphoplasmacellular infiltrates rich in IgG4-positive cells and fibrotic bile duct strictures are characteristic features of this disease. IgG4-SC can be successfully treated with corticosteroids.

Methods: A full text search of the database of the Department of Histopathology's for the term „klatskin tumor“ was performed. All cases without malignant findings and with features of sclerosing cholangitis were reviewed and immunohistochemically stained for IgG4 positive plasma cells.

Results: Ten patients who underwent hepatobiliary surgery for suspected cholangiocarcinoma (CCC) between 2002 and 2009 were retrospectively identified as IgG4-related sclerosing cholangitis by IgG4-staining of resected liver tissue. Preoperatively, all patients (8 men and 2 women, mean age 59,5 years) presented with asymptomatic jaundice and a distinct elevation of liver function tests (mean ALT 199 U/L, AP 471U/l, Gamma-GT 651U/l, bilirubin 84µmol/L). Suspicion of CCC was based on endoscopic findings and hepatobiliary imaging studies. Intraoperatively, no malignancy was found in any case. Performed surgical procedures were hemihepatectomies in seven cases (6 right sided) with 2 consecutive liver transplantions for small for size hepatectomy, two biliodigestive anastomosis and one liver transplantation. After retrospective identification of IgG4-SC in these patients, they were followed up in 2010. At that time, 2 patients still presented with signs of active sclerosing cholangitis and received corticosteroids and/or PTCD. Another patient was treated with corticosteroids for weight loss and lymphadenopathy. One patient died during follow-up and 3 were lost to follow up. The other 3 patients had no signs of ongoing IgG4-related sclerosing disease.

Conclusion: IgG4-related sclerosing cholangitis is a recently recognized important differenzial diagnosis of cholangiocarcinoma. It can mislead to major liver surgery. Post-operative recurrence is possible necessitating medical or endoscopic therapy.