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Case Reports
. 2015 Jul;9(4):556-60.
doi: 10.5009/gnl14241.

IgG4-Associated Cholangitis Can Mimic Hilar Cholangiocarcinoma

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Free PMC article
Case Reports

IgG4-Associated Cholangitis Can Mimic Hilar Cholangiocarcinoma

Victor M Zaydfudim et al. Gut Liver. .
Free PMC article

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.

Keywords: Autoimmune cholangiopathy; Autoimmune pancreatitis; Biliary stricture; Hilar mass; IgG4 cholangiopathy.

Figures

Fig. 1
Fig. 1
MR contrast-enhanced T1-weighted image (A) demonstrating a 1-cm hilar mass (arrow) with a MR cholangiogram (B) demonstrating a corresponding hilar biliary stricture (arrow).
Fig. 2
Fig. 2
MR contrast-enhanced T1-weighted image (A) demonstrating a 2.1-cm hilar mass (arrow) with a MR cholangiogram (B) demonstrating a corresponding hilar biliary stricture (arrow).
Fig. 3
Fig. 3
Histologic features of IgG4-associated cholangitis. (A) Common bile duct with dense mucosal inflammation, transmural inflammation and fibrosis (H&E stain, ×20; inset: magnification of mucosal inflammatory infiltrate, showing the predominance of plasma cells with occasional eosinophils, ×400). (B) Fibrosis of the common bile duct wall. Thick collagen bundles are present with intervening spindled fibroblasts and lymphocytes (H&E stain, ×200). (C) Neurovascular bundle within the common bile duct wall with lymphoplasmacytic infiltration of a nerve (N) and a small vein (V) (H&E stain, ×100). (D) Immunohistochemical stain for IgG4, demonstrating a high density of IgG4-positive plasma cells (brown stain) in the common bile duct mucosa (×200).

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