Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis
10.15279/kpba.2025.30.2.87
- Author:
Dong Wook KIM
1
;
Hee Yoon NOH
;
Song-Hee HAN
;
Myung Hwan NOH
Author Information
1. Division of Gastroenterology, Department of Internal Medicine, Dong-A University Hospital, Busan, Korea
- Publication Type:Case Report
- From:Korean Journal of Pancreas and Biliary Tract
2025;30(2):87-92
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
We report a rare case of overlapping immunoglobulin G4-sclerosing cholangitis (IgG4-SC) and primary biliary cholangitis (PBC) in a 78-year-old woman presenting with sudden-onset jaundice, cholestatic liver enzyme elevation, and biliary strictures. Elevated alkaline phosphatase levels, positive antimitochondrial antibodies, increased serum IgG4 levels, and a high IgG4/IgG ratio, combined with endobiliary biopsy via endoscopic retrograde cholangiopancreatography biopsy showing lymphoplasmacytic infiltration and IgG4-positive cells, confirmed the diagnosis. Initial treatment with biliary drainage, ursodeoxycholic acid (UDCA), and corticosteroids provided temporary relief; however, the patient experienced recurrent episodes of cholangitis and biliary obstruction, eventually progressing to decompensated cirrhosis over 2 years. This case highlights the importance of considering IgG4-SC in patients with PBC who fail to respond adequately to UDCA and underscores the significant challenges in diagnosing and managing such overlapping syndromes. Further research is crucial to better understand the underlying pathophysiology, refine therapeutic strategies, and improve clinical outcomes of these rare, complex autoimmune conditions.
