IgG4-associated cholangitis can mimic hilar cholangiocarcinoma

Victor M. Zaydfudim*, Andrew Y. Wang, Eduard E. De Lange, Zimin Zhao, Christopher A. Moskaluk, Todd W. Bauer, Reid B. Adams

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

13 Scopus citations


IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.

Original languageEnglish
Pages (from-to)556-560
Number of pages5
JournalGut and Liver
Issue number4
StatePublished - 1 Jul 2015
Externally publishedYes


  • Autoimmune cholangiopathy
  • Autoimmune pancreatitis
  • Biliary stricture
  • Hilar mass
  • IgG4 cholangiopathy


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