平均肺动脉压力可作为结缔组织病相关肺间质病的预后指标

作者：Takahashi K 翻译：金银姬 校对：武东

摘要：背景：肺动脉高压可见于结缔组织病相关间质性肺病（CTD-ILD）患者，并可导致结缔组织病相关间质性肺病发病率和死亡率的增高。然而，除对系统性硬化症和混合性结缔组织病外，平均肺动脉压力（MPAP）对生存率的影响尚未充分阐明。我们推测肺动脉压力可能为结缔组织病相关间质性肺病的预后因素，与结缔组织病种类无关。

方法：我们评估了平均肺动脉压力对于多种合并间质性肺病的结缔组织病患者生存的影响。回顾性分析了所纳入研究的结缔组织病相关间质性肺病患者数据。研究对象均在初始评估时行肺功能检查及右心导管检查。

结果：共纳入74名患者（33名男性，41名女性，平均年龄为62.8?±?9.6岁，其中24名类风湿关节炎，14名系统性硬化症，14名多发性肌炎/皮肌炎，11名原发性干燥综合征，11名其他诊断）。结果显示，6名患者合并肺高压(平均肺动脉压力?≥?25 mmHg)，16名(21.6 %)患者合并平均肺动脉压力轻度升高（平均肺动脉压力 (≥20 mmHg)）。平均平均肺动脉压力为17.2?±?5.5 mmHg。研究发现，多种结缔组织病患者之间平均肺动脉压力无显著差异。单变量Cox比例风险模型显示，平均肺动脉压力对生存率有显著影响，但结缔组织病具体类型对于生存率无影响。多变量Cox比例风险模型显示平均肺动脉压力（HR?=?1.087；95% CI 1.008-1.172；P = 0.030??）为生存的独立决定因素。

结论： 平均肺动脉压力轻度升高常见于有多种结缔组织病背景的结缔组织病相关间质性肺病患者。初始评估时平均肺动脉压力较高为结缔组织病相关间质性肺病患者生存的独立预测因素。平均肺动脉压力的评估可提供更多关于疾病状态的信息，帮助医生预测结缔组织病相关间质性肺病的死亡率。

附原文：Abstract BACKGROUND: Pulmonary hypertension (PH) can develop in connective tissue disease associated interstitial lung disease (CTD-ILD), and contributes to increased morbidity and mortality. However, except for systemic sclerosis and mixed connective tissue disease, the impact of mean pulmonary arterial pressure (MPAP) on survival in CTD-ILD has not been sufficiently elucidated. We hypothesized that pulmonary arterial pressure may be a prognostic factor in CTD-ILDs regardless of the kind of CTD. METHODS: We evaluated the survival impact of MPAP, which is measured using right heart catheterization, on survival of patients with CTD-ILD with various CTD backgrounds. We retrospectively analyzed data of consecutive CTD-ILD patients undergoing a pulmonary function test and right-heart-catheterization at the initial evaluation. RESULTS: We studied 74 patients (33 men and 41 women, mean age 62.8?±?9.6, 24 with rheumatoid arthritis, 14 with systemic sclerosis, 14 with polymyositis/dermatomyositis, 11 with primary Sj?gren's syndrome, and 11 with other diagnoses). Six patients exhibited pulmonary hypertension (MPAP?≥?25 mmHg), and 16 (21.6 %) had mild elevation of MPAP (≥20 mmHg). The mean MPAP was 17.2?±?5.5 mmHg. We did not observe a significant difference in MPAP among various CTDs. A univariate Cox proportional hazard model showed that MPAP has a significant impact on survival, while the type of CTD did not contribute to survival in our cohort. A multivariate Cox proportional hazard model showed MPAP (HR?=?1.087; 95 % CI 1.008-1.172; p?=?0.030) to be the sole independent determinant of survival. CONCLUSIONS: Mild elevation of MPAP is relatively common in CTD-ILD patients with various CTD backgrounds. A higher MPAP at the initial evaluation was a significant independent predictor of survival in CTD-ILD. MPAP evaluation provides additional information on disease status and will help physicians predict mortality in CTD-ILD.

引自：Takahashi K, Taniguchi H, Ando M. BMC Pulm Med. Mean pulmonary arterial pressure as a prognostic indicator in connective tissue disease associated with interstitial lung disease: a retrospective cohort study. 2016 Apr 19;16(1):55. doi: 10.1186/s12890-016-0207-3.