Primary biliary cholangitis: Diagnosis and treatment

Abstract

Primary biliary cholangitis (PBC) is an autoimmune disease that causes progressive destruction of the intrahepatic bile ducts. Although the pathogenesis of PBC is not completely understood, it is characterized by cholestatic inflammation resulting in a spectrum of cholangitis, varying degrees of fibrosis, and, potentially, cirrhosis. PBC affects all races; however, there have been few clinical studies of the disease in Asian countries, particularly in Southeast Asia, suggesting that PBC may be underreported in the Asian population. Here, we review the epidemiology, natural history, predictive and diagnostic features, and current and emerging treatments for PBC. Our goal is also to highlight current studies from Asian countries to provide a better understanding of PBC in this patient population. Keywords: Primary biliary cholangitis (PBC), Epidemiology, Natural history, Diagnosis, Treatment