IGG4 Related Hepatobiliary Disease: A Role for ERCP

Aims IGG4 related hepatobiliary disease can present with symptomatic biliary obstruction alongside cholangiopathy and pseudotumours. We describe a cohort of patients undergoing Endoscopic Retrograde Cholangiopancreatography (ERCP) prior to diagnosis of IGG4 related hepatobiliary disease (IGG4-RD) and report on their clinical course.

Methods Consecutive patients with IGG4-RD who underwent ERCP over a five year period were identified. Patients were diagnosed based on histology, imaging and serum IGG4 levels.

Results Thirteen patients were identified. The median age at diagnosis was 64 years old (range 28-85 years old). None had sites of IGG4 involvement outside the hepatobiliary system. Nine (69.2 %) had both intra and extrahepatic involvement, two (15.4 %) each had intrahepatic strictures or common bile duct strictures alone. Four (30.8 %) presented with pseudo-tumours.

Histology was obtained via endoscopic ultrasound (EUS) in 10 patients (76.9 %) and via ERCP with intraductal biopsies in three (23.1 %). Median IGG4 levels at diagnosis was 4.54 (range 0.73 -12.48). All had fully covered self-expanding metal stents (FC-SEMS) inserted.

Eleven (84.6 %) patient received Prednisolone for induction therapy. Three (23.1 %) patients had Prednisolone monotherapy for maintenance, four (30.8 %) had dual therapy with Azathioprine and one received Rituximab (intolerant of Prednisolone and Azathioprine due to side effects). Four (30.8 %) were not on maintenance immunosuppression and two (15.4 %) patients had stricture resolution with placement of FC-SEMS alone. All are still under follow up. All had marked improvement in biliary strictures following treatment and are currently in remission.

Conclusions We describe a cohort of patient with IGG4 related hepatobiliary disease. We show that in this setting EUS guided biopsy alongside ERCP is an effective method of tissue acquisition. We corroborate previous findings of improvement with medical therapy and show that ERCP can be a useful adjunct.

Citation Ahmed W, Karim S, Sidhu B et al. eP369 IGG4 RELATED HEPATOBILIARY DISEASE: A ROLE FOR ERCP. Endoscopy 2021; 53: S216.

Publication History

Article published online:
19 March 2021