Suzuki Atsushi, Taniguchi Hiroyuki, Watanabe Naohiro, Kondoh Yasuhiro, Kimura Tomoki, Kataoka Kensuke, Matsuda Toshiaki, Yokoyama Toshiki, Sakamoto Koji, Nishiyama Osamu, Hasegawa Yoshinori
Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.
Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.
PLoS One. 2014 Sep 30;9(9):e108339. doi: 10.1371/journal.pone.0108339. eCollection 2014.
Lung-dominant connective tissue disease (LD-CTD) is a new concept for classifying the subset of patients with interstitial pneumonia who have clinical features suggesting an associated CTD, but whose features fall short of a clear diagnosis of CTD under the current rheumatologic classification systems. The impact of mean pulmonary arterial pressure (MPAP) in LD-CTD has not been sufficiently elucidated.
To evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.
We retrospectively analyzed the initial evaluation data of 100 LD-CTD patients undergoing pulmonary function test, 6-min walk test (6MWT), and right heart catheterization (RHC).
The mean MPAP was 16.2±4.4 mm Hg, and 18 patients had MPAP≥20 mm Hg. A univariate Cox proportional hazard model showed that MPAP and several variables have a statistically significant impact on survival. With stepwise, multivariate Cox proportional analysis, MPAP (HR = 1.293; 95% CI 1.130-1.480; p<0.001) and mean forced vital capacity (FVC) % predicted (HR = 0.958; 95% CI 0.930-0.986; p = 0.004) were shown to be independent determinants of survival.
Higher MPAP and lower %FVC at the initial evaluation were significant independent prognostic factors of LD-CTD. MPAP evaluation provides additional information of disease status and will help physicians to predict mortality in LD-CTD.
肺部为主的结缔组织病（LD-CTD）是一个新的概念，用于对间质性肺炎患者亚组进行分类，这些患者具有提示相关结缔组织病的临床特征，但根据当前的风湿病分类系统，其特征不足以明确诊断为结缔组织病。平均肺动脉压（MPAP）在LD-CTD中的影响尚未得到充分阐明。
评估在LD-CTD患者初始评估时测量的MPAP对生存的影响。
我们回顾性分析了100例接受肺功能测试、6分钟步行试验（6MWT）和右心导管检查（RHC）的LD-CTD患者的初始评估数据。
平均MPAP为16.2±4.4 mmHg，18例患者MPAP≥20 mmHg。单变量Cox比例风险模型显示，MPAP和几个变量对生存有统计学显著影响。通过逐步多变量Cox比例分析，MPAP（HR = 1.293；95%CI 1.130 - 1.480；p<0.001）和预测的平均用力肺活量（FVC）百分比（HR = 0.958；95%CI 0.930 - 0.986；p = 0.004）被证明是生存的独立决定因素。
初始评估时较高的MPAP和较低的FVC百分比是LD-CTD的重要独立预后因素。MPAP评估提供了疾病状态的额外信息，并将帮助医生预测LD-CTD的死亡率。
