迟发型结缔组织病相关肺动脉高压的临床特征及预后分析

doi:10.3969/j.issn.1003-9198.2026.08.016

摘要/Abstract

摘要： 目的探索迟发型结缔组织病相关肺动脉高压(CTD-PAH)患者的临床特征及预后不良的危险因素。方法回顾性分析2019年1月至2023年12月期间于南京医科大学第一附属医院风湿免疫科诊治且由右心导管诊断的CTD-PAH患者的临床资料。根据发病年龄分为早发型组(发病年龄<60岁)和迟发型组(发病年龄≥60岁)。比较2组患者人口学资料、心血管疾病危险因素(高血压、冠心病、糖尿病)、二维超声心动图参数、右心导管参数、原发病与肺动脉高压(PAH)治疗及无临床失败事件生存率等,并分析临床失败事件的影响因素。结果与早发型组相比,迟发型组冠心病及高血压患病率显著增高,心功能状态更差,NYHA心功能Ⅲ~Ⅳ级比例更高,6 min步行距离(6MWD)显著缩短(P＜0.05)。二维超声心动图示:迟发型组左心房内径(LAD)、左心室舒张末内径(LVDd)及E/e′均较早发型组显著增加(P＜0.05)。迟发型组糖皮质激素使用率及PAH联合靶向治疗率均显著低于早发型组(P＜0.05)。随访1年内,迟发型组治疗达标率较早发型组更低(25.0%比62.1%,P＜0.05)。Cox回归分析提示,E/e′比值是迟发型患者发生临床失败事件的危险因素(HR=1.295,95%CI:1.039~1.615,P=0.022) 。结论迟发型CTD-PAH患者具有较高的心血管疾病危险因素发生率,存在左心房压力升高和左心室舒张功能受损,总体预后较差,E/e′是该类患者不良预后的危险因素。

关键词: 迟发型, 结缔组织病, 肺动脉高压, 临床特征, 预后

Abstract: Objective To explore the clinical characteristics and risk factors for poor prognosis in patients with late-onset connective tissue disease (CTD) associated pulmonary arterial hypertension (PAH). Methods A retrospective analysis was conducted on patients diagnosed with CTD-PAH at Department of Rheumatology, the First Affiliated Hospital of Nanjing Medical University from January 2019 to December 2023 using right heart catheterization(RHC). Patients were divided into two groups: early-onset group(onset age<60 years) and late-onset group(onset age ≥60 years). Demographic data, cardiovascular risk factors (hypertension, coronary artery disease and diabetes), echocardiographic and RHC parameters, treatment for CTD and PAH, clinical failure-free survival rates were observed and compared between the two groups. And the influecing factors for clinical failve events was analysized. Results Compared with the early-onset group, the late-onset group had a higher prevalence of coronary heart disease and hypertension; Late-onset patients exhibited worse cardiac function, with a higher proportion of cardiac function grading Ⅲ-Ⅳ and significantly shorter 6-minute walk distance (6MWD) (P＜0.05). Two-dimensional echocardiography showed that left atrial diameter (LAD), left ventricular end-diastolic diameter (LVDd), and the E/e′ ratio were significantly increased in the late-onset group than those in the early-onset group(P＜0.05). Compared with the early-onset group, the utilization rate of glucocorticoids and the rate of combination targeted therapy for PAH were significantly lower in the late-onset group(P＜0.05). Within one year of follow-up, the proportion of patients achieving a low-risk status in the late-onset group was significantly lower than that in the early-onset group (25.0% vs 62.1%,P＜0.05). Cox regression analysis indicated that the E/e′ ratio was a risk factor for clinical failure events in patients with late-onset CTD-PAH(HR=1.295,95%CI:1.039-1.615,P=0.022). Conclusions Late-onset CTD-PAH patients have a higher incidence of cardiovascular risk factors, with elevated left atrial pressure and impaired left ventricular diastolic function. They generally have a poorer prognosis, and E/e′ is a risk factor for adverse outcomes in these patients.

Key words: late-onset, connective tissue disease, pulmonary arterial hypertension, clinical characteristics, prognosis

中图分类号:

许洁, 张译心, 张缪佳, 王嫱, 孙晓萱. 迟发型结缔组织病相关肺动脉高压的临床特征及预后分析[J]. 实用老年医学, 2026, 40(8): 837-842.

XU Jie, ZHANG Yixin, ZHANG Miaojia, WANG Qiang, SUN Xiaoxuan. Clinical characteristics and prognostic analysis of late-onset connective tissue disease-associated pulmonary arterial hypertension[J]. Practical Geriatrics, 2026, 40(8): 837-842.

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