《中国肺动脉高压诊断与治疗指南(2021版)》解读——结缔组织病相关肺动脉高压.pdfVIP

《中国肺动脉高压诊断与治疗指南（2021版）》解读 ——结缔组织病相关肺动脉高压 钱君岩，赵久良，王 迁，李梦涛，曾小峰 摘要：肺动脉高压（PH）是结缔组织病（CTD）的严重并发症之一，其中，以第一大类PH，即动脉性肺动脉高压（PAH）最 为常见。因起病隐匿、临床表现缺乏特异性、早期诊断困难、治疗效果不佳，PAH已成为CTD患者死亡的重要因素之 一。文章针对中华医学会呼吸病学分会肺栓塞与肺血管病学组发布的《中国肺动脉高压诊断与治疗指南（2021版）》 中CTD相关PAH部分的推荐意见进行解读，提倡CTD相关PAH的早期筛查、精确诊断、全面评估和精准治疗，以期 改善CTD相关PAH患者的长期预后，提高患者生活质量。 关键词：结缔组织病；肺动脉高压；系统性红斑狼疮；干燥综合征；系统性硬化症；双重达标策略 中图分类号：R543.2 文献标识码：A Interpretation of the Guidelines to the diagnosis and treatment of pulmonary hypertension in China （（2021））: pulmonaryhypertensionassociatedwithconnectivetissuedisease. QIANJun-yan，ZHAOJiu-liang，WANGQian， LIMeng-tao，ZENGXiao-feng.DepartmentofRheumatologyandClinicalImmunology,ChineseAcademyofMedical SciencesPekingUnionMedicalCollege,NationalClinicalResearchCenterforDermatologicandImmunologicDiseases (NCRC-DID),MinistryofScienceTechnology,StateKeyLaboratoryofComplexSevereandRareDiseases,PekingUnion MedicalCollegeHospital(PUMCH),KeyLaboratoryofRheumatologyandClinicalImmunology,MinistryofEducation, Beijing100730,China Correspondingauthor:LIMeng-tao，E-mail:mengtao.li@cstar.org.cn Abstract: Pulmonaryhypertension （PH）isoneoftheseriouscomplicationsofconnectivetissuedisease （CTD）.Among them，thefirsttypeofPH，namelyarterialpulmonaryhypertension （PAH），isthemostcommon.Duetotheinsidious onset，lackofspecificclinicalmanifestations，difficultyinearlydiagnosis，andpoortreatmenteffects，PAHhasbecome oneoftheimportantfactorsinthedeathofCTDpatients.ThisarticleinterpretstherecommendationsoftheCTD-related PAHsectionoftheGuidelinestothediagnosisandtreatmentofpulmonaryhypertensioninChina（2021）issuedbythe PulmonaryEmbolismandPulmonaryVascularDiseaseGroupoftheRespiratoryDiseasesBranchoftheChineseMedical Association，andpromoteearlyscreening，accuratediagnosis，comprehensiveevaluationandprecisetreatmentofCTD-related PAHinordertoimprovethelong-termprognosisofpatientswithCTD-relatedPAHandimprovethequalityoflifeofpatients. Keywords:connectivetissuedisease;pulmonaryh