Progress and Perspectives in Overlapping Primary Biliary Cholangitis and Autoimmune Hepatitis: A Comprehensive Review

Abstract

Overlapping primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) represents a distinctive autoimmune phenotype characterized by concurrent cholestatic and hepatocellular damage, culminating in a more aggressive disease course if not recognized. This review synthesizes the existing evidence concerning epidemiology, pathophysiology, and diagnostic criteria, with particular emphasis on histopathology, serological markers, and established scoring systems, including the Paris criteria and the International Autoimmune Hepatitis Group (IAIHG) scoring scheme. We highlight the clinical relevance of combined therapies, typically comprising ursodeoxycholic acid and immunosuppressants, in effectively controlling both components of the disorder and halting fibrosis progression. Additionally, we discuss emerging data regarding second-line agents such as obeticholic acid and innovative immunomodulatory approaches aimed at refining patient outcomes. Special attention is dedicated to pediatric and pregnant populations, in whom disease manifestation and therapeutic responses may differ. Ongoing evaluations of noninvasive biomarkers and imaging modalities, including transient elastography, promise improved monitoring and individualized management strategies. Notably, relevant outcome measures, including quality of life and the burden of pruritus, are paramount for comprehensive patient care. Collectively, these advances hold promise for improved long-term patient survival by enabling more precise diagnostic pathways, targeted therapeutic regimens, and closer surveillance.