1Liver Institute Northwest, Seattle, Washington, USA.
2UC Davis Health, Sacramento, California, USA.
3Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, Miami, Florida, USA.
4Utah Southwestern Medical Center, Dallas, Texas, USA.
5Massachusetts General Hospital, Boston, Massachusetts, USA.
6Indiana University School of Medicine, Indianapolis, Indiana, USA.
7Inova Fairfax Medical Campus, Falls Church, Virginia, USA.
Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune liver disease that can progress to end-stage liver disease and its complications. A previous expert review panel collaborated on a consensus document for gastroenterologists and other healthcare professionals regarding the care of patients with PBC. Subsequently, there have been several recent important developments in the diagnosis, treatment, and monitoring of patients with PBC. These include updates to prognostic models on risk stratification, new noninvasive tools for staging of disease, updates to the appropriate use of and long-term treatment results with obeticholic acid as a second-line treatment, the emerging therapeutic role of fibrates, and the advancement of investigational agents for managing PBC. In this updated expert consensus document, we provide updates on staging, the use of noninvasive prognostic tools, and a treatment algorithm to provide evidence-based and practical tools for clinicians who manage PBC, with the ultimate goal to improve the long-term outcomes for patients with this chronic liver disease.