实用临床医药杂志2026，Vol.30Issue(17)：39-45,7.DOI:10.7619/jcmp.20262209
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
Application of fourtier risk stratification model in prognostic assessment of patients with connective tissue disease associated pulmonary arterial hypertension in China
摘要
Abstract
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.关键词
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Key words
underlying disease/connective tissue disease/pulmonary arterial hypertension/fourtier risk stratification model/right heart catheterization/systemic lupus erythematosus/primary Sjögren syndrome分类
医药卫生引用本文复制引用
韩笑,郭珂珺,许洁,孙晓萱,周媛媛..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[J].实用临床医药杂志,2026,30(17):39-45,7.基金项目
国家自然科学基金资助项目(81701610) （81701610）
