Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune liver disease characterized by progressive destruction of small intrahepatic bile ducts. If left untreated, PBC may eventually result in end-stage liver disease. For better management of PBC and optimal allocation of medical resources, it is pivotal to accurately estimate the prognosis of patients with PBC. This article will briefly review the models that predict long-term outcome of PBC patients, with special focus on the applicability, strengths and limitations of the widely used models reported from 1983 to 2016. Among many, the Mayo score has been extensively validated and considered as the classic prognostic model for untreated PBC patients, whereas the well-validated Paris I and Paris II criteria are mainly used in ursodeoxycholic acid (UDCA)-treated patients with advanced PBC (stage III-IV) and early PBC (stege I-II), respectively. Based on multicenter studies with large sample sizes, the recently reported GLOBE score and UK-PBC score seem to be superior to previous models and can be applied in patients with different stages of PBC who are already on UDCA therapy, but further external validation may be justified.
Keywords: Primary biliary cholangitis; Primary biliary cirrhosis; Prognosis; Prognostic model; Score.