Abstract
IgG4-sclerosing cholangitis (IgG4-SC) commonly presents with type 1 autoimmune pancreatitis. Isolated IgG4-SC is rare. Differentiating IgG4-SC from cholangiocarcinoma preoperatively is challenging due to overlapping radio-clinical manifestations and difficult preoperative histology. We present three cases preoperatively diagnosed and surgically treated as hilar cholangiocarcinoma. First and second cases presented with cholangiocarcinoma with portal vein involvement and third with a malignant-appearing hilar stricture. On histopathology, IgG4-SC was diagnosed in the first two cases. Third patient had raised serum IgG4, and histopathology was inconclusive for IgG4-SC and negative for malignancy. However, she responded to steroid therapy.
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References
Stone JH, Brito-Zerón P, Bosch X, Ramos-Casals M. Diagnostic approach to the complexity of IgG4-related disease. Mayo Clin Proc. 2015;90:927–39.
Shimosegawa T, Chari ST, Frulloni L, et al. International consensus diagnostic criteria for autoimmune pancreatitis: guidelines of the International Association of Pancreatology. Pancreas. 2011;40:352–8.
Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology. 2008;134:706–15.
Ozer Cakir O, Ataseven H, Biyik M, Gungor G, Demir A. Autoimmune cholangiopathy followed with the diagnosis of cholangiocarcinoma. EJOHG. 2012;2:119–21.
Nakazawa T, Naitoh I, Hayashi K, Miyabe K, Simizu S, Joh T. Diagnosis of IgG4-related sclerosing cholangitis. World J Gastroenterol. 2013;19:7661–70.
Miki A, Sakuma Y, Ohzawa H, et al. Immunoglobulin g4-related sclerosing cholangitis mimicking hilar cholangiocarcinoma diagnosed with following bile duct resection: report of a case. Int Surg. 2015;100:480–5.
Zaydfudim VM, Wang AY, de Lange EE, et al. IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Gut Liver. 2015;9:556–60.
Lytras D, Kalaitzakis E, Webster GJ, et al. Cholangiocarcinoma or IgG4-associated cholangitis: how feasible it is to avoid unnecessary surgical interventions? Ann Surg. 2012;256:1059–67.
Gonzalez S, Moreira RK, Verna EC, Samstein B, Poneros JM. Immunoglobulin g4 associated cholangitis can mimic cholangiocarcinoma on radiologic and cholangioscopic findings. Gastroenterol Hepatol. 2013;9:252–4.
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Yadav, K.S., Sali, P.A., Mansukhani, V.M. et al. IgG4-associated sclerosing cholangitis masquerading as hilar cholangiocarcinoma. Indian J Gastroenterol 35, 315–318 (2016). https://doi.org/10.1007/s12664-016-0672-x
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DOI: https://doi.org/10.1007/s12664-016-0672-x