原发性硬化性胆管炎诊疗指南（2025年版）
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Guidelines for the diagnosis and treatment of primary sclerosing cholangitis （2o25 edition）
Abstract：Inorder to further standardize thediagnosisandtreatmentofrarediseasesandensure medicalqualityand safety， HealthCommisionofthePeople’sRepublicofChinadevelopedtheguidelinesforthediagnosisandtreatmentof 86 diseases intheSecondListofRare Diseases，whichwereoffciallreleasedinJune2O25，includingfiverarehepatobliarydiseases ofAlagillesyndrome，αl-antitrypsindeficiency，congenitalbiliaryatresia，primarybiliarycholangitis，andprimarysclerosing cholangitis.Thisarticleintroducestheetiology，epidemilogy，clinicalmanifestations，auxiliaryexamination，diagnosis，and treatment of primary sclerosing cholangitis，in order to provideareference for clinical practice.
KeyWords：Cholangitis，Sclerosing；Diagnosis；Therapeutics；Practice Guideline
2025年6月，发布了《第二批罕见病目录》中86个病种的诊疗指南[1]，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硬化性胆管炎（primarysclerosingcholangitis，PSC）的相关内容，供临床参考。（剩余6839字）
