Takahashi Kota, Taniguchi Hiroyuki, Ando Masahiko, Sakamoto Koji, Kondoh Yasuhiro, Watanabe Naohiro, Kimura Tomoki, Kataoka Kensuke, Suzuki Atsushi, Ito Satoru, Hasegawa Yoshinori
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
Department of Respiratory Medicine and Allergy, Tosei General Hospital, 160 Nishioiwake-cho, Seto, Aichi, 489-8642, Japan.
BMC Pulm Med. 2016 Apr 19;16(1):55. doi: 10.1186/s12890-016-0207-3.
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.
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.
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 Sjögren'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.
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.
肺动脉高压（PH）可在结缔组织病相关间质性肺病（CTD-ILD）中发生，并导致发病率和死亡率增加。然而，除系统性硬化症和混合性结缔组织病外，平均肺动脉压（MPAP）对CTD-ILD患者生存的影响尚未得到充分阐明。我们假设，无论CTD的类型如何，肺动脉压可能是CTD-ILD的一个预后因素。
我们评估了通过右心导管测量的MPAP对不同CTD背景的CTD-ILD患者生存的影响。我们回顾性分析了在初始评估时接受肺功能测试和右心导管检查的连续CTD-ILD患者的数据。
我们研究了74例患者（33例男性和41例女性，平均年龄62.8±9.6岁，24例类风湿关节炎，14例系统性硬化症，14例多发性肌炎/皮肌炎，11例原发性干燥综合征，11例其他诊断）。6例患者出现肺动脉高压（MPAP≥25mmHg），16例（21.6%）MPAP轻度升高（≥20mmHg）。平均MPAP为17.2±5.5mmHg。我们未观察到不同CTD之间MPAP有显著差异。单变量Cox比例风险模型显示，MPAP对生存有显著影响，而CTD类型对我们队列中的生存无影响。多变量Cox比例风险模型显示MPAP（HR = 1.087；95%CI为1.008 - 1.172；p = 0.030）是生存的唯一独立决定因素。
MPAP轻度升高在不同CTD背景的CTD-ILD患者中相对常见。初始评估时较高的MPAP是CTD-ILD患者生存的显著独立预测因素。MPAP评估提供了有关疾病状态的额外信息，并将帮助医生预测CTD-ILD患者的死亡率。
