A Rare Case of Autoimmune Hepatitis-Primary Biliary Cholangitis Overlap Syndrome in a Male Patient.
Publication Title
Cureus
Document Type
Article
Publication Date
1-1-2025
Keywords
washington; olympia; psph
Abstract
Autoimmune liver diseases, such as autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), present significant diagnostic and therapeutic challenges due to overlapping features and potential for severe complications. AIH-PBC overlap syndrome, a rare condition, combines characteristics of both diseases but lacks standardized treatment protocols. We present the case of a 42-year-old male with elevated liver function tests, pruritus, flatulence, and epigastric pain. Laboratory findings revealed a cholestatic liver function pattern, a highly positive antinuclear antibody titer, weakly positive anti-smooth muscle antibody, elevated immunoglobulin G, and negative viral and anti-mitochondrial antibody markers. Diagnostic imaging, including abdominal ultrasound and magnetic resonance cholangiopancreatography, demonstrated mild fatty liver, slight irregularity in the left biliary duct wall, and a normal common bile duct without significant abnormalities. Liver biopsy confirmed chronic hepatitis with dense portal lymphoplasmacytic infiltrate, scattered eosinophils, moderate interface hepatitis, and mild lobular necroinflammation, consistent with AIH-PBC overlap syndrome. The Paris criteria were used to establish the diagnosis. Treatment with ursodeoxycholic acid, prednisolone, and azathioprine resulted in significant clinical and biochemical improvement.
Area of Special Interest
Digestive Health
Specialty/Research Institute
Internal Medicine
Specialty/Research Institute
Gastroenterology
Specialty/Research Institute
Hepatology
DOI
10.7759/cureus.77023
