Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases

Guidance Statements 9 and 10 that were published in the 2018 Practice Guidance on primary biliary cholangitis (PBC) have been revised.[1] This American Association for the Study of Liver Diseases 2021 practice guidance on PBC is a focused update of the PBC guidance published in 2018. The major changes from the last guidance to this guidance include information about obeticholic acid (OCA). In May 2021, the Food and Drug Administration issued a new warning restricting the use of OCA in patients with advanced cirrhosis.[2] This is defined as cirrhosis with current or prior evidence of liver decompensation (e.g., encephalopathy, coagulopathy) or portal hypertension (e.g., ascites, gastroesophageal varices, or persistent thrombocytopenia). Two guidance statements that were published in the 2018 practice guidance on PBC have been revised as follows: 9. Fibrates can be considered as off-label alternatives for patients with PBC and inadequate response to ursodeoxycholic acid, although fibrates are discouraged in patients with decompensated liver disease. 10. OCA is contraindicated in patients with advanced cirrhosis. This is defined as cirrhosis with current or prior evidence of liver decompensation (e.g., encephalopathy, coagulopathy) or portal hypertension (e.g., ascites, gastroesophageal varices, or persistent thrombocytopenia). Furthermore, we would recommend careful monitoring of any patient with cirrhosis, even if not advanced, receiving OCA. The authors are grateful for the valuable contributions of the American Association for the Study of Liver Diseases Practice Guideline Committee. Members of the committee include George Ioannou (chair); Rabab Ali, Scott W. Biggins, Roniel Cabrera, Henry Chang, Michael F. Chang, Albert Do, David S. Goldberg, R. W. Ray Kim (board liaison), Lindsay Y. King, Cynthia Levy, Jeff McIntyre, Jessica L. Mellinger, Anthony J. Michaels, Mindie H. Nguyen, Nadia Ovchinsky, Anjana A. Pillai, Daniel S. Pratt, Hugo R. Rosen, Christopher J. Sonnenday, Matthew J. Stotts, Lisa B. VanWagner, and Elizabeth C. Verna. Dr. Mayo consults for and received grants from Cymabay. She received grants from Intercept. Dr. Levy consults for, advises, and received grants from Intercept, Cymabay, Genfit, GSK, Pliant, Mirum, and Cara Therapeutics. She consults for and advises Escient, Teva, and Calliditas. She received grants from Gilead, Novartis, High Tide, Zydus, and Target PharmaSolutions. She receives royalities from Up-to-date.