Significance of Pulmonary Arterial Pressure as a Prognostic Indicator in Lung-Dominant Connective Tissue Disease |
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Authors: | Atsushi Suzuki Hiroyuki Taniguchi Naohiro Watanabe Yasuhiro Kondoh Tomoki Kimura Kensuke Kataoka Toshiaki Matsuda Toshiki Yokoyama Koji Sakamoto Osamu Nishiyama Yoshinori Hasegawa |
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Affiliation: | 1. Department of Respiratory Medicine and Allergy, Tosei General Hospital, Seto, Aichi, Japan.; 2. Department of Respiratory Medicine, Nagoya University Graduate School of Medicine, Nagoya, Aichi, Japan.; 3. Department of Respiratory Medicine and Allergology, Kinki University Faculty of Medicine, Osaka-sayama, Osaka, Japan.; Keio University School of Medicine, Japan, |
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Abstract: | BackgroundLung-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.ObjectivesTo evaluate the survival impact of MPAP measured during the initial evaluation in patients with LD-CTD.MethodsWe 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).ResultsThe 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.ConclusionsHigher 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. |
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