AASLD AGA clinical Practice Guideline on management of primary biliary cholangitis

Abstract

Background & Aims: Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease associated with progressive fibrosis, cirrhosis, liver transplantation, and premature mortality. Despite effective therapies, important challenges remain in the diagnosis and management of PBC, including identification of antimitochondrial antibody–negative disease, assessment of response to ursodeoxycholic acid (UDCA), risk stratification, treatment of patients with inadequate response, management of cholestatic pruritus, and prevention of recurrent disease after liver transplantation. The aim of this guideline was to develop evidence-based recommendations addressing these key areas of clinical care. Methods: The American Gastroenterological Association (AGA) and the American Association for the Study of Liver Diseases (AASLD) convened a multidisciplinary panel to develop clinical practice recommendations for adults with PBC. Systematic reviews were performed for 6 prioritized clinical questions. The Grading of Recommendations Assessment, Development and Evaluation framework was used to prioritize clinical outcomes and assess evidence, and its Evidence to Decision Framework was used to develop recommendations. Results: The guideline panel agreed on 6 recommendations. These recommendations address the use of PBC-specific antinuclear antibodies in antimitochondrial antibody–negative suspected PBC, UDCA as first-line therapy, liver stiffness measurement for prognostication, peroxisome proliferator–activated receptor agonists for patients with inadequate response or intolerance to UDCA, linerixibat for moderate to severe pruritus, and UDCA prophylaxis post-transplantation to reduce disease recurrence. The certainty of evidence ranged from moderate to very low. Conclusions: The guideline emphasizes early diagnosis, optimization of first-line therapy, risk-based treatment escalation, symptom management, and prevention of post-transplantation disease recurrence to improve patient outcomes and quality of life.