四分层风险评估模型对中国结缔组织病相关肺动脉高压患者预后的应用价值OA
Application of fourtier risk stratification model in prognostic assessment of patients with connective tissue disease associated pulmonary arterial hypertension in China
目的 回顾性分析结缔组织病相关肺动脉高压(CTD-PAH)患者的临床特征、预后,并验证四分层风险评估模型在随访中的预后评估价值.方法 回顾性纳入2009年10月—2023年10月于南京医科大学第一附属医院经右心导管检查(RHC)确诊的172例CTD-PAH患者.收集人口学特征、原发病种类、病程、PAH评估参数(NT-proBNP、WHO心功能分级、6分钟步行试验、RHC及心脏超声参数)和治疗方案,并根据2022年欧洲心脏病学会/欧洲呼吸学会(ESC/ERS)指南,采用COMPERA 2.0方法进行四分层危险评估.主要终点事件为5年全因死亡.采用Kaplan-Meier法绘制生存曲线.结果 CTD-PAH原发病以系统性红斑狼疮(SLE)和原发性干燥综合征(pSS)为主.CTD-PAH患者1年、3年和5年总生存率分别为93.5％(95％CI:89.7％～97.5％)、82.7％(95％CI:76.0％～90.0％)和 77.1％(95％CI:69.1％～86.0％).不同原发病患者预后差异显著,系统性硬化病相关PAH(SSc-PAH)预后最差(5年生存率52.9％).99例完成首次随访四分层评估的患者中,高危、中高危、中低危及低危组的3年生存率分别为0％、46.7％、75.0％和96.3％(P＜0.05);治疗达标者3年生存率优于未达标者,差异有统计学意义(P＜0.05).结论 CTD-PAH最常见的原发病为SLE和pSS,SSc-PAH患者预后极差.四分层模型能精准识别高危人群,可作为指导CTD-PAH全程管理及治疗决策的有效工具.
Objective To retrospectively analyze the clinical characteristics and prognosis of pa-tients with connective tissue disease associated pulmonary arterial hypertension(CTD-PAH),and to validate the prognostic value of a fourtier risk stratification model during follow-up.Methods A total of 172 patients with CTD-PAH diagnosed by right heart catheterization(RHC)at the First Affiliated Hospital of Nanjing Medical University between October 2009 and October 2023 were retrospectively enrolled.Demographic characteristics,type of underlying CTD,disease duration,PAH related as-sessment parameters(NT-proBNP,WHO functional class,6 minute walk test,RHC and echocardio-graphic parameters),and treatment regimens were collected.According to the 2022 European Society of Cardiology/European Respiratory Society(ESC/ERS)guidelines,the COMPERA2.0 method was used to perform a fourtier risk stratification.The primary endpoint was 5 year all cause mortality.Survival curves were generated using the Kaplan-Meier method.Results The most common underlying dis-ease of CTD-PAH were systemic lupus erythematosus(SLE)and primary Sjögren syndrome(pSS).The 1,3,and 5 year overall survival rates of CTD-PAH patients were 93.5％(95％CI:89.7％-97.5％),82.7％(95％CI:76.0％-90.0％),and 77.1％(95％CI:69.1％-86.0％),respectively.Survival differed significantly across underlying CTD subtypes,with systemic sclerosis associated PAH(SSc-PAH)carrying the worst prognosis(5 year survival rate:52.9％).Among 99 patients who completed the fourtier risk assessment at the first follow-up,the 3 year survival rates for the highrisk,intermediate high risk,intermediate low risk,and low risk groups were 0％,46.7％,75.0％,and 96.3％,respectively(P＜0.05).Patients who achieved treatment targets had significantly better 3 year survival than those who did not(P＜0.05).Conclusion SLE and pSS are the most common underlying disease of in CTD-PAH,whereas SSc-PAH carries an extremely poor prognosis.The fourtier risk stratification model can accurately identify high risk patients and may serve as an effective tool for guiding whole course management and treatment deci-sion-making in CTD-PAH.
韩笑;郭珂珺;许洁;孙晓萱;周媛媛
南京医科大学第一附属医院风湿免疫科,江苏南京,210029南京医科大学附属明基医院,江苏南京,210019南京医科大学第一附属医院风湿免疫科,江苏南京,210029南京医科大学第一附属医院风湿免疫科,江苏南京,210029南京医科大学第一附属医院风湿免疫科,江苏南京,210029
医药卫生
原发病结缔组织病肺动脉高压四分层风险评估模型右心导管检查系统性红斑狼疮原发性干燥综合征
underlying diseaseconnective tissue diseasepulmonary arterial hypertensionfourtier risk stratification modelright heart catheterizationsystemic lupus erythematosusprimary Sjögren syndrome
《实用临床医药杂志》 2026 (17)
39-45,7
国家自然科学基金资助项目(81701610)
评论