Diagnosis and treatment of primary biliary cholangitis: Current status and challenges

Primary biliary cholangitis is an autoimmune liver disease often observed in women, with the main features of positive antimitochondrial antibodies in serum and progressive non-pyogenic inflammatory destruction of small intrahepatic bile ducts. At present, primary biliary cholangitis is considered the result of interaction between chronic immune injury and biliary epithelium under the combined effect of inheritance and environment. Ursodeoxycholic acid is the first-line drug for primary biliary cholangitis; for patients who cannot tolerate ursodeoxycholic acid or have suboptimal response, the second-line drug obeticholic acid, an FXR agonist, can be considered, and the peroxisome proliferator-activated receptor agonists fibrates can be regarded as second-line alternative drugs. Baseline characteristics (such as young age, male sex, and advanced disease) and blood biochemical parameters (especially bilirubin and alkaline phosphatase) are used for disease risk stratification and response evaluation. Management of the symptoms such as pruritus and weakness cannot be ignored.