原发性胆汁性胆管炎诊疗指南（2025年版）

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DOI：10.12449/JCH250805

Guidelines for the diagnosis and treatment of primary biliary cholangitis （2o25 edition）

National Health Commissionof thePeople'sRepublic ofChina

Abstract：Inorder to further standardize the diagnosisand treatmentofrarediseasesandensure medicalqualityand safety， NationalHealthCommisionofthePeople’sRepublicofChinadevelopedtheguidelines forthediagnosisandtreatmentof86 diseases intheSecondListofRare Diseases，whichwereoffciallyreleasedinJuneO25，includingfiverarehepatobliarydiseases ofAlagillesyndrome，α-antitrypsindefiiency，congenitalbiliaryatresia，primarybiliarycholangitis，andprimaryslerosing cholangitis.Thisarticleintroduces theetiology，epidemilogy，clinical manifestations，auxiliaryexamination，diagnosis，and treatment of primary biliary cholangitis，in order to provide a reference for clinical practice.

KeyWords：Primary Biliary Cholangitis；Diagnosis；Therapeutics；Practice Guideline

2025年6月，中华人民共和国国家卫生健康委员会发布了《第二批罕见病目录》中86个病种的诊疗指南[，旨在进一步提高我国罕见病诊疗规范化水平，本文特摘录其中关于原发性胆汁性胆管炎（primarybiliarycholangitis，PBC）的相关内容，供临床参考。（剩余3975字）