IgG4-related sclerosing cholangitis involving the gallbladder mimicking a hilar cholangiocarcinoma

A 76-year-old man presented with abdominal pain, jaundice, and fever. Abdominal computed tomography demonstrated wall thickening of the gallbladder with gallstones surrounding a large low-density lesion communicating with the gallbladder and a thickened, enhancing wall at the hepatic hilum and common hepatic duct (CHD). Magnetic resonance cholangiopancreatography (MRCP) showed a stricture at the hilum extending to the CHD, suggestive of a hilar cholangiocarcinoma ([Fig. 1]). Percutaneous catheter drainage for liver abscess and endoscopic retrograde cholangiopancreatography with stent placement for biliary drainage and biopsy was performed. Histology did not show malignancy. Same-session endoscopic ultrasound (EUS)-guided tissue acquisition and peroral cholangioscopy (POCS) were performed. EUS revealed symmetrical and smooth wall thickening of the hilum and CHD. EUS-guided tissue acquisition was performed using a 22-gauge needle ([Fig. 2]). POCS (SpyGlass DS; Boston Scientific, Natick, Massachusetts, USA) demonstrated a stricture with dilated vessels and hyperemic and papillary-appearing mucosa in the hilum and CHD ([Video 1]). Specimens were obtained using the SpyBite biopsy forceps (Boston Scientific). The pathology results from EUS-guided tissue acquisition and SpyBite forceps biopsy showed stromal fibrosis with lymphoplasmacytic infiltration and more than 10 IgG4-positive plasma cells per high-power field (HPF) ([Fig. 3]). Serum IgG4 level was 185 mg/dL. Laparoscopic cholecystectomy was performed, and on histology the wall of the gallbladder showed multifocal lymphoplasmacytic infiltrations with more than 10 IgG4-positive plasma cells per HPF. The patient received steroid treatment at a dosage of 40 mg/day. After 4 weeks of steroid treatment, MRCP demonstrated improved luminal narrowing of the hilum and CHD ([Fig. 4]). Therefore, IgG4-related sclerosing cholangitis involving the gallbladder was diagnosed. The patient was placed on long-term low-dose steroid treatment, the biliary stent was removed, and the patient has now had no recurrence of the cholangitis for over 1 year.

Fig. 1 Magnetic resonance cholangiopancreatography (MRCP) showing bile duct stenosis (arrowheads) in the hepatic hilum extending to the common hepatic duct, suggestive of a hilar cholangiocarcinoma.

Fig. 2 Endoscopic ultrasound (EUS) showing symmetrical and smooth wall thickening (thin arrows) of the hepatic hilum and CHD, and EUS-guided tissue acquisition using a 22-gauge needle (open arrow) was performed for the biliary lesion.

Video 1 Peroral cholangioscopy reveals a stricture with dilated vessels, a hyperemic, edematous mucosal surface, and a papillary-appearing mucosal projection. SpyBite forceps biopsy specimens were taken from the abnormal mucosal lesions.

Fig. 3 Histology of endoscopic ultrasound-guided fine-needle aspiration/biopsy and SpyBite forceps biopsy: a stromal fibrosis with lymphoplasmacytic infiltration (H&E, × 200); b more than 10 IgG4-positive plasma cells per high-power field (IgG4 stain, × 400).

Fig. 4 MRCP 4 weeks after steroid treatment showed an improved state of the hilar biliary stricture (arrow).

IgG4-related sclerosing cholangitis is difficult to differentiate from malignancy [1]. EUS-TA and POCS may be a useful modality for evaluating an indeterminate hilar stricture [2] [3].

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Publication History

Article published online:
10 March 2022

• References

• 1 Naitoh I, Nakazawa T. Classification and diagnostic criteria for IgG4-related sclerosing cholangitis. Gut Liver 2022; 16: 28-36
• 2 de Moura DTH, Ryou M, de Moura EGH. et al. Endoscopic ultrasound-guided fine needle aspiration and endoscopic retrograde cholangiopancreatography-based tissue sampling in suspected malignant biliary strictures: a meta-analysis of same-session procedures. Clin Endosc 2020; 53: 417-428
• 3 Parsa N, Khashab MA. The role of peroral cholangioscopy in evaluating indeterminate biliary strictures. Clin Endosc 2019; 52: 556-564