Abstract：
BACKGROUND: The pathogenesis and clinical profiles of patients with pulmonary hypertension (PH) associated with interstitial lung disease (ILD-PH) are poorly understood. Whether and to what extent pulmonary arterial hypertension (PAH)-specific therapy improves hemodynamic and outcome in ILD-PH are also unknown.
OBJECTIVE: This study aims to clarify the characteristics, clinical course and response to PAH-specific therapy of ILD and/or PH by enrolling three unique subsets: PAH, ILD-PH, and ILD.
METHODS: The proposed study is a retrospective and prospective, multi-centre, observational cohort study of patients treated at any of three university hospitals in the Hokkaido region of Japan who have any one of the following: PAH; ILD-PH with or without PAH features; or ILD without PH. We aim to enrol 250 patients in total. For the retrospective observation period, data obtained after 1 January 2010, will be analysed, and the prospective observation period will be 1 year. We will compare the clinical data of patients with ILD-PH with those of patients with PAH and those of patients with ILD without PH in the real-world clinical setting. In addition, within the cohort of patients with ILD-PH, we will explore the subset with \"ILD-PH with PAH features\" and compare the response to PAH-specific therapy with that of PAH. The primary outcome will be the change in pulmonary vascular resistance from first treatment to follow-up in patients with PAH and ILD-PH with PAH features (excluding ILD-PH without PAH feature and ILD-no-PH for the primary outcome). The exploratory outcomes will include analyses of PH-associated biomarkers, right ventricular function and patient-reported outcomes.
RESULTS: This is a protocol article and the results will be presented after data collection is completed.
CONCLUSIONS: The POPLAR study will provide data that help better understand the pathophysiology of ILD-PH and improve the quality of life and outcome of patients with PH and/or ILD.
BACKGROUND: Japan Registry of Clinical Trials: jRCT1010230018.
OBJECTIVE: This study aims to clarify the characteristics, clinical course and response to PAH-specific therapy of ILD and/or PH by enrolling three unique subsets: PAH, ILD-PH, and ILD.
METHODS: The proposed study is a retrospective and prospective, multi-centre, observational cohort study of patients treated at any of three university hospitals in the Hokkaido region of Japan who have any one of the following: PAH; ILD-PH with or without PAH features; or ILD without PH. We aim to enrol 250 patients in total. For the retrospective observation period, data obtained after 1 January 2010, will be analysed, and the prospective observation period will be 1 year. We will compare the clinical data of patients with ILD-PH with those of patients with PAH and those of patients with ILD without PH in the real-world clinical setting. In addition, within the cohort of patients with ILD-PH, we will explore the subset with \"ILD-PH with PAH features\" and compare the response to PAH-specific therapy with that of PAH. The primary outcome will be the change in pulmonary vascular resistance from first treatment to follow-up in patients with PAH and ILD-PH with PAH features (excluding ILD-PH without PAH feature and ILD-no-PH for the primary outcome). The exploratory outcomes will include analyses of PH-associated biomarkers, right ventricular function and patient-reported outcomes.
RESULTS: This is a protocol article and the results will be presented after data collection is completed.
CONCLUSIONS: The POPLAR study will provide data that help better understand the pathophysiology of ILD-PH and improve the quality of life and outcome of patients with PH and/or ILD.
BACKGROUND: Japan Registry of Clinical Trials: jRCT1010230018.
摘要：
背景：与间质性肺病（ILD-PH）相关的肺动脉高压（PH）患者的发病机制和临床特征知之甚少。肺动脉高压（PAH）特异性治疗是否以及在多大程度上改善了ILD-PH的血液动力学和结果也未知。
目的：本研究旨在阐明其特征，通过纳入三个独特的子集，对ILD和/或PH的PAH特异性治疗的临床过程和反应：PAH，ILD-PH,和ILD。
方法：拟议的研究是一项回顾性和前瞻性的研究，多中心,在日本北海道地区的三家大学医院接受治疗的患者的观察性队列研究，这些患者患有以下任何一种：PAH;ILD-PH有或没有PAH特征；或ILD无PH。我们的目标是招募250名患者。对于回顾性观察期，2010年1月1日之后获得的数据将进行分析，前瞻性观察期为1年。我们将在真实世界的临床环境中比较ILD-PH患者与PAH患者和无PHILD患者的临床数据。此外,在ILD-PH患者队列中，我们将探索具有“ILD-PH具有PAH特征”的亚组,并比较PAH特异性治疗与PAH的反应.主要结果将是具有PAH特征的PAH和ILD-PH患者从首次治疗到随访的肺血管阻力变化（不包括无PAH特征的ILD-PH和无ILD-PH的主要结果）。探索性结果将包括对PH相关生物标志物的分析，右心室功能和患者报告的结果。
结果：这是一篇协议文章，结果将在数据收集完成后呈现。
结论：POPLAR研究将提供有助于更好地了解ILD-PH的病理生理学并改善PH和/或ILD患者的生活质量和预后的数据。
背景：日本临床试验注册：jRCT1010230018。
目的：本研究旨在阐明其特征，通过纳入三个独特的子集，对ILD和/或PH的PAH特异性治疗的临床过程和反应：PAH，ILD-PH,和ILD。
方法：拟议的研究是一项回顾性和前瞻性的研究，多中心,在日本北海道地区的三家大学医院接受治疗的患者的观察性队列研究，这些患者患有以下任何一种：PAH;ILD-PH有或没有PAH特征；或ILD无PH。我们的目标是招募250名患者。对于回顾性观察期，2010年1月1日之后获得的数据将进行分析，前瞻性观察期为1年。我们将在真实世界的临床环境中比较ILD-PH患者与PAH患者和无PHILD患者的临床数据。此外,在ILD-PH患者队列中，我们将探索具有“ILD-PH具有PAH特征”的亚组,并比较PAH特异性治疗与PAH的反应.主要结果将是具有PAH特征的PAH和ILD-PH患者从首次治疗到随访的肺血管阻力变化（不包括无PAH特征的ILD-PH和无ILD-PH的主要结果）。探索性结果将包括对PH相关生物标志物的分析，右心室功能和患者报告的结果。
结果：这是一篇协议文章，结果将在数据收集完成后呈现。
结论：POPLAR研究将提供有助于更好地了解ILD-PH的病理生理学并改善PH和/或ILD患者的生活质量和预后的数据。
背景：日本临床试验注册：jRCT1010230018。
