Tanabe Nobuhiro, Kumamaru Hiraku, Tamura Yuichi, Kondoh Yasuhiro, Nakayama Kazuhiko, Kinukawa Naoko, Kimura Tomoki, Nishiyama Osamu, Tsujino Ichizo, Shigeta Ayako, Morio Yoshiteru, Inoue Yoshikazu, Kuraishi Hiroshi, Hirata Ken-Ichi, Tanaka Kensuke, Kuwana Masataka, Nagaoka Tetsutaro, Handa Tomohiro, Sugimura Koichiro, Sakamaki Fumio, Naito Akira, Taniguchi Yu, Matsubara Hiromi, Hanaoka Masayuki, Inami Takumi, Hayama Naoki, Nishimura Yoshihiro, Kimura Hiroshi, Miyata Hiroaki, Tatsumi Koichiro
Pulmonary Hypertension Center, Saiseikai Narashino Hospital, Narashino, Chiba, Japan.
Department of Respirology, Graduate School of Medicine, Chiba University, Chiba, Japan.
JACC Asia. 2024 Mar 26;4(5):403-417. doi: 10.1016/j.jacasi.2024.01.009. eCollection 2024 May.
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.
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.
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.
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.
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).
近期指南不鼓励在患有与呼吸系统疾病相关的肺动脉高压（PAH）的患者中使用PAH靶向治疗。因此，对PAH靶向治疗的有效性进行分层对该群体很重要。
作者旨在确定在患有间质性肺炎以及合并肺纤维化和肺气肿的肺动脉高压（PH）患者中可能从初始PAH靶向治疗中获益的表型。
我们使用5个伍德单位（WU）的肺血管阻力将270例毛细血管前PH患者（192例间质性肺炎，78例合并肺纤维化和肺气肿）分为重度和轻度PH。我们研究了预后因素，并比较了初始（诊断后2个月内）和非初始治疗组的预后，以及反应者（世界卫生组织功能分级、肺血管阻力和6分钟步行距离改善）和无反应者。
在239例未接受过治疗的患者中，46.0%为重度PH，51.8%有轻度通气功能障碍（VI），40.6%接受了初始治疗。在重度PH合并轻度VI亚组中，初始治疗组与非初始治疗组相比预后较好。该组的反应率显著高于其他组（48.2%对21.8%，比值2.21 [95%CI：1.17 - 4.16]）。在多变量分析中，初始治疗是重度PH的更好预后因素，但不是轻度PH的预后因素。在重度PH亚组中，反应者预后较好。
本研究表明，初始PAH靶向治疗的反应者数量增加，在重度PH合并轻度VI的病例中预后良好。在轻度PH病例中未观察到生存获益。（与呼吸系统疾病相关的肺动脉高压多机构前瞻性登记研究；UMIN000011541）
