Natural History of Primary Biliary Cholangitis in the Ursodeoxycholic Acid Era Role of Scoring Systems

Primary biliary cholangitis (PBC) is a chronic disease that progresses to end-stage liver disease. Ursodeoxycholic acid (UDCA), the standard treatment for PBC for several decades, is associated with improved survival without liver transplant. Approximately 40% of patients do not respond to UDCA. Because of disease variability, there exist several prognostic models that incorporate various factors including biochemical response to UDCA. These models are useful for patient care and counseling as well as risk stratification for research and clinical trials, and the role of these models in the pre-UDCA and UDCA eras is discussed.