Follicular Cholangitis: A Rare Cause of Benign Biliary Stricture

Virginia Lampropoulou; Athanasios Sioulas; Konstantina Papadaki; Spyridon Pagkratis; Dimitrios Papaioannou; George Tzimas; Ilias Scotiniotis

doi:10.12890/2023_004152

Follicular Cholangitis: A Rare Cause of Benign Biliary Stricture

Eur J Case Rep Intern Med. 2023 Nov 13;10(12):004152. doi: 10.12890/2023_004152. eCollection 2023.

Authors

Virginia Lampropoulou¹, Athanasios Sioulas¹, Konstantina Papadaki¹, Spyridon Pagkratis², Dimitrios Papaioannou³, George Tzimas², Ilias Scotiniotis¹

Affiliations

¹ 3 Department of Gastroenterology, Hygeia Hospital, Athens, Greece.
² Department of Hepatobiliary Surgery, Hygeia Hospital, Athens, Greece.
³ Pathology Laboratory, Hygeia Hospital, Athens, Greece.

Abstract

Introduction: The differential diagnosis of focal biliary strictures comprises both malignant and benign conditions. We report a rare case of follicular cholangitis presenting with segmental stricture of the left hepatic duct.

Case description: An asymptomatic 59-year-old male with no past medical history presented with dilation of the left intrahepatic bile ducts revealed as an incidental finding on an abdominal ultrasound. Blood examinations showed only a slightly elevated γ-glutamyl transferase (γGT) value, while carbohydrate antigen 19-9 (Ca 19-9) and serum immunoglobulin G4 (IgG4) were within normal range. Abdominal computed tomography and magnetic resonance imaging/magnetic resonance cholangiopancreatography (MRI/MRCP) scans revealed a high grade focal intrahepatic stricture of the left hepatic duct (FIHS type III) with proximal dilatation. Given that a diagnosis of cholangiocarcinoma could not be ruled out, the patient was referred for a left hepatectomy with regional lymph node dissection. Histological analysis showed a lymphoplasmacytic infiltration of the left hepatic duct with fibrosis and follicle formations in the submucosa, findings consistent with follicular cholangitis. The postoperative course was uneventful and there is no evidence of recurrence 8 months after the surgery.

Discussion: The clinical and imaging presentation of follicular cholangitis is very similar to cholangiocarcinoma, rendering it a challenging diagnosis preoperatively.

Conclusion: The approach to these cases should be primarily surgical. Even though it is very rare -- our report is the 13^th case reported worldwide -- follicular cholangitis should be included in the differential diagnosis of focal biliary strictures.

Learning points: The differential diagnosis of biliary strictures comprises malignancies, like cholangiocarcinoma, as well as benign conditions.It is very challenging to distinguish between malignant and benign biliary strictures preoperatively, so the most reliable treatment approach to these cases is often surgical.Follicular cholangitis is a very rare condition and more data is needed to better understand disease pathophysiology, management, recurrence rates, and possible alternatives to surgery.

Keywords: Follicular cholangitis; biliary strictures; cholangiocarcinoma.