Nobuhiro Tanabe, Hiraku Kumamaru, Yuichi Tamura, Yasuhiro Kondoh, Kazuhiko Nakayama, Naoko Kinukawa, Tomoki Kimura, Osamu Nishiyama, Ichizo Tsujino, Ayako Shigeta, Yoshiteru Morio, Yoshikazu Inoue, Hiroshi Kuraishi, Ken-Ichi Hirata, Kensuke Tanaka, Masataka Kuwana, Tetsutaro Nagaoka, Tomohiro Handa, Koichiro Sugimura, Fumio Sakamaki, Akira Naito, Yu Taniguchi, Hiromi Matsubara, Masayuki Hanaoka, Takumi Inami, Naoki Hayama, Yoshihiro Nishimura, Hiroshi Kimura, Hiroaki Miyata, Koichiro Tatsumi
JACC. Asia 4(5) 403-417 2024年5月
BACKGROUND: Recent guidelines discourage the use of pulmonary arterial hypertension (PAH)-targeted therapies in patients with pulmonary hypertension (PH) associated with respiratory diseases. Therefore, stratifications of the effectiveness of PAH-targeted therapies are important for this group. OBJECTIVES: The authors aimed to identify phenotypes that might benefit from initial PAH-targeted therapies in patients with PH associated with interstitial pneumonia and combined pulmonary fibrosis and emphysema. METHODS: We categorized 270 patients with precapillary PH (192 interstitial pneumonia, 78 combined pulmonary fibrosis and emphysema) into severe and mild PH using a pulmonary vascular resistance of 5 WU. We investigated the prognostic factors and compared the prognoses of initial (within 2 months after diagnosis) and noninitial treatment groups, as well as responders (improvements in World Health Organization functional class, pulmonary vascular resistance, and 6-minute walk distance) and nonresponders. RESULTS: Among 239 treatment-naive patients, 46.0% had severe PH, 51.8% had mild ventilatory impairment (VI), and 40.6% received initial treatment. In the severe PH with mild VI subgroup, the initial treatment group had a favorable prognosis compared with the noninitial treatment group. The response rate in this group was significantly higher than the others (48.2% vs 21.8%, ratio 2.21 [95% CI: 1.17-4.16]). In multivariate analysis, initial treatment was a better prognostic factor for severe PH but not for mild PH. Within the severe PH subgroup, responders had a favorable prognosis. CONCLUSIONS: This study demonstrated an increased number of responders to initial PAH-targeted therapy, with a favorable prognosis in severe PH cases with mild VI. A survival benefit was not observed in mild PH cases. (Multi-institutional Prospective Registry in Pulmonary Hypertension associated with Respiratory Disease; UMIN000011541).