结缔组织病相关性肺动脉高压是否合并肺间质疾病的临床特征与预后分析

Clinical characteristics and prognosis of pulmonary arterial hypertension associated with connective tissue diseases with or without interstitial lung disease

摘要:

目的比较是否合并间质性肺病(interstitial lung disease, ILD)的结缔组织病相关肺动脉高压(pulmonary arterial hypertension associated with connective tissue diseases, CTDs-PAH)的临床特征及其评估预后的价值，并对比其在各个类型中的差异。 方法纳入2010年1月—2023年12月在同济大学附属上海市肺科医院经右心导管检查明确诊断CTDs-PAH患者共301例。将所有患者分为不合并ILD组(n=225)和合并ILD组(n=76)两组，比较两组患者的临床特征差异，应用Cox回归分析死亡风险增加的相关因素。对比CTDs-PAH三种常见病因[系统性红斑狼疮组(SLE-PAH,n=141)、干燥综合征组(pSS-PAH,n=70)、系统性硬化症组(SSc-PAH,n=32)]的差异。 结果合并ILD的CTDs-PAH患者发病年龄平均为(47.60±7.13)岁，明显高于无ILD组(P＜0.001)。74%合并ILD的CTDs-PAH患者在初始诊断时WHO-FC就已达到Ⅲ/Ⅳ级(P=0.028)，6-MWD更差(P＜0.001)。合并ILD的患者FVC%、FEV1%、TLC%更低(P＜0.001)，弥散功能更差(P=0.001)。Cox回归分析结果提示，WHO-FC[HR(95%CI)为2.906(1.333~6.335)，P=0.007]、合并ILD[HR(95%CI)为0.458(0.234~0.897)，P=0.023]、DLCO[HR(95%CI)为0.737(0.560~0.971)，P=0.030]是CTDs-PAH的独立死亡风险因素。40.6%的SSc-PAH合并ILD，明显高于SLE-PAH(12.5%)和pSS-PAH(28.6%)(P＜0.001)。SSc-PAH的DLCO明显低于SLE-PAH(P＜0.001)和pSS-PAH(P＜0.001)。SLE-PAH合并ILD最少，SaO2也高于pSS-PAH(P=0.009)、SSc-PAH(P=0.015)。 结论WHO-FC Ⅲ/Ⅳ、合并ILD和DLCO升高预测CTDs-PAH患者死亡的独立风险因素。早期完善胸部HRCT及肺功能检查，及早干预纠正肺间质病变能改善患者预后。

Abstract:

ObjectiveTo compare the clinical characteristics of connective tissue disease-associated pulmonary hypertension(CTDs-PAH) with or without interstitial lung disease(ILD) and its value in prediction of prognosis. MethodsA total of 301 CTDs-PAH patients hospitalized in the Shanghai Pulmonary Hospital of Affiliated to Tongji University from January 2010 to December 2023 were collected. All the patients were diagnosed via right heart catheterization. All the patients were divided into two groups: the non-ILD group(n=225) and the ILD group(n=76). The differences in clinical characteristics between the two groups were compared, and Cox regression analysis was applied to determine the factors related to the increased risk of death. Compare the differences of clinical characteristics among the three most common types of CTDs-PAH [systemic lupus erythematosus group(SLE-PAH, n=141), Sjogrens syndrome group(pSS-PAH, n=70), and systemic sclerosis group(SSc-PAH, n=32)]. ResultsThe average age of onset for CTDs-PAH patients with ILD was(47.60±7.13) years, it was significantly higher than that in patients without ILD(P<0.001). Seventy-four percent of CTDs-PAH patients with ILD had already reached WHO-FC Ⅲ/Ⅳ at the initial diagnosis(P=0.028), 6-MWD was even worse(P<0.001). Patients with ILD had lower FVC%, FEV1%, and TLC%(all P<0.001), and poorer diffusion function(P=0.001). The results of Cox regression analysis indicated that WHO-FC [HR(95%CI) was 2.906(1.333-6.335), P=0.007] and combined with ILD[HR(95%CI) was 0.458(0.234-0.897), P=0.023] and DLCO [HR(95%CI) was 0.737(0.560-0.971), P=0.030] were independent risk factors for death. ILD was presented in 40.6% of SSc-PAH, which was significantly higher than that of SLE-PAH(12.5%) and pSS-PAH(28.6%)(P<0.001). The DLCO of SSc-PAH was significantly lower than that of SLE-PAH and pSS-PAH(both P<0.001). SLE-PAH was the least likely to be combined with ILD, and the SaO2 was also higher than that in pSS-PAH(P=0.009) and SSc-PAH(P=0.015). ConclusionWHO-FC Ⅲ/Ⅳ, combined with ILD and elevated DLCO are independent risk factors for predicting death in patients with CTDs-PAH. Early completion of chest HRCT and pulmonary function tests, as well as early intervention of ILD, can enhance clinical outcomes.