Korean J Pancreas Biliary Tract.  2018 Jul;23(3):127-133. 10.15279/kpba.2018.23.3.127.

A 2-Week Steroid Trial for Differentiating Isolated IgG4-Related Sclerosing Cholangitis from Cholangiocarcinoma

Affiliations
  • 1Department of Internal Medicine, Dongansan Hospital, Ansan, Korea.
  • 2Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea. endomoon@naver.com
  • 3Department of Pathology, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea.

Abstract

Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is rare disease which is steroid-responsive and often associated with IgG4 related systemic disease such as autoimmune pancreatitis. It is characterized by increased serum IgG4 and IgG4-positive lymphoplasmacytic infiltration in bile ducts. It is often difficult to distinguish IgG4-SC to hilar cholangiocarcinoma if it manifests as an isolated bile duct. We report a case of 79-year-old woman with IgG4-SC which was difficult to distinguish hilar cholangiocarcinoma due to similar clinical and radiologic findings, showing good therapeutic effect after a 2-week steroid trial.

Keyword

Immunoglobulin G4; Sclerosing cholangitis; Steroid; Cholangiocarcinoma
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