Gut Liver.  2015 Jul;9(4):556-560. 10.5009/gnl14241.

IgG4-Associated Cholangitis Can Mimic Hilar Cholangiocarcinoma

• Zaydfudim
• Wang AY
• de Lange E
• Zhao Z
• Moskaluk C
• Bauer TW
• Adams RB

• KMID: 2160641
• DOI: http://doi.org/10.5009/gnl14241

Abstract

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.

Keyword

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

MeSH Terms

Aged
Bile Ducts/pathology/surgery
Cholangitis/blood/*diagnosis
Diagnosis, Differential
Humans
Immunoglobulin G/*blood
Klatskin Tumor/blood/*diagnosis
Liver/pathology/surgery
Male
Immunoglobulin G