IgG4-related sclerosing cholangitis suspected of being malignant tumors: A case series

Abstract

BACKGROUND IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IRD). IgG4-SC often has the characteristics of localized thickening of the bile duct wall, dilation of the bile duct, and obstructive jaundice, which are similar to those of biliary and pancreatic malignancies and often lead to delayed diagnosis or even misdiagnosis clinically. AIM To improve the clinicians’ understanding of IgG4-SC and avoid its misdiagnosis by analyzing the clinical characteristics, diagnosis, and treatment of five cases of IgG4-SC. METHODS A retrospective analysis was performed on five cases of IgG4-SC suspected of being space-occupying lesions of the porta hepatis, ampulla, and pancreas at admission. Patient information, including age, gender, symptoms, signs, past medical history, and the process of diagnosis and treatment, was collected. RESULTS Four cases showed obstructive jaundice and imaging identified one case of high level biliary obstruction and three cases of low level obstruction, which suggested the possibility of malignancy. The obstruction sites were located in the porta hepatis, pancreatic head, or ampulla. One case was found to have a mass at the neck of the pancreas by physical examination. All five patients had bile duct inflammation, swelling of the pancreas, or space-occupying lesions in the imaging examination. Laboratory examinations revealed elevated bilirubin, mainly direct bilirubin, accompanied by liver dysfunction. Serum IgG4 levels were significantly increased and tumor markers were normal or slightly elevated. Four patients were diagnosed with IgG4-SC by multidisciplinary consultation, and one patient was diagnosed by pathological examination after surgery. CONCLUSION Multidisciplinary consultation is an important strategy in diagnosing IgG4-SC and avoiding misdiagnosis.