Isolated IgG4-related sclerosing cholangitis misdiagnosed as malignancy in an area with endemic cholangiocarcinoma: a case reportReportar como inadecuado

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BMC Surgery

, 17:17

Case reports


BackgroundThe most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma.

Case presentationWe report the case of a 56-year-old man who presented with obstructive jaundice with preoperative imaging showing proximal common bile duct obstruction. He underwent right lobe liver hepatectomy with extrahepatic bile duct resection and regional lymph node dissection due to high suspicion of malignancy. The pathological report showed severe acute and chronic inflammation of the bile duct with morphology and immunohistochemistry suggestive of IgG4-related sclerosing cholangitis.

ConclusionsIgG4-related sclerosing cholangitis with perihilar obstruction should be considered even in areas where cholangiocarcinoma is endemic.

KeywordsIgG4, IgG4-related sclerosing cholangitis Cholangiocarcinoma Benign biliary stricture Sclerosing cholangitis AbbreviationsAIPAutoimmune pancreatitis

ALPAlkaline phosphatase

ALTAlanine aminotransferase

ASTAspartate aminotransferase

CA 19–9Cancer antigen 19–9


CEACarcinoembryonic antigen

CTComputed tomography

ERCPEndoscopic retrograde cholangiopancreatography

GGTGamma glutamyl transferase

HAVHepatitis A virus

HBsAgHepatitis B surface antigen

HCVHepatitis C virus

IDUSIntraductal ultrasound

IgG4-RDIgG4-related disease

IgG4-SCIgG4-sclerosing cholangitis

NPVNegative predictive value

PPVPositive predictive value

SOPCSingle-Operator Peroral Cholangioscopy

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Autor: Narongsak Rungsakulkij - Pattana Sornmayura - Penampai Tannaphai


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