Korean J Pancreas Biliary Tract.  2025 Apr;30(2):87-92. 10.15279/kpba.2025.30.2.87.

Liver Cirrhosis Progression in a Patient with Overlapping IgG4-Related Sclerosing Cholangitis and Primary Biliary Cholangitis

• Kim DW
• Noh HY
• Han SH
• Noh MH

• KMID: 2567428
• DOI: http://doi.org/10.15279/kpba.2025.30.2.87

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.

Keyword

Bile duct diseases; Primary biliary cholangitis; IgG4-related disease; IgG4-related sclerosing disease; Cholangiopancreatography; endoscopic retrograde; 담관 질환; 원발성 담즙성 담관염; IgG4 연관 질환; IgG4 연관 경화성 담관염; 내시경적역행성담췌관조영술