A PrOsPective Cohort Study on Interstitial Lung Disease-Associated Pulmonary Hypertension with a ParticulaR Focus on the Subset with Pulmonary Arterial Hypertension Features (POPLAR Study).

IF 2.3 Q2 RESPIRATORY SYSTEM

Pulmonary Therapy Pub Date : 2024-09-01 Epub Date: 2024-06-24 DOI:10.1007/s41030-024-00264-0

Ichizo Tsujino, Kazuki Kitahara, Junichi Omura, Toshiyuki Iwahori, Satoshi Konno

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Abstract

Introduction: 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.

Study 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.

Conclusion: 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.

Trial registration: Japan Registry of Clinical Trials: jRCT1010230018.

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间质性肺病相关性肺动脉高压的队列研究，重点关注具有肺动脉高压特征的人群（POPLAR 研究）。

导言：人们对肺动脉高压（PAH）伴间质性肺病（ILD-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能改善 ILD-PH 患者的血液动力学和预后也不得而知：本研究旨在通过纳入三个独特的子集，阐明 ILD 和/或 PH 的特征、临床过程以及对 PAH 特异性治疗的反应：研究目的：本研究旨在通过纳入三个独特的子集：PAH、ILD-PH 和 ILD，明确 PAH 特异性治疗的临床过程和反应：拟议的研究是一项回顾性和前瞻性、多中心、观察性队列研究，研究对象是在日本北海道地区三家大学医院中接受治疗的患者，这些患者患有以下任何一种疾病：PAH；具有或不具有 PAH 特征的 ILD-PH；或不具有 PH 特征的 ILD。我们的目标是招募 250 名患者。回顾性观察期将分析 2010 年 1 月 1 日之后获得的数据，前瞻性观察期为 1 年。我们将把 ILD-PH 患者的临床数据与 PAH 患者和无 PH 的 ILD 患者的临床数据进行比较。此外，在 ILD-PH 患者队列中，我们还将探索 "具有 PAH 特征的 ILD-PH 患者 "子集，并比较 PAH 患者与 PAH 患者对 PAH 特异性治疗的反应。主要结果将是 PAH 患者和具有 PAH 特征的 ILD-PH 患者从首次治疗到随访期间肺血管阻力的变化（主要结果不包括无 PAH 特征的 ILD-PH 和 ILD-no-PH）。探索性结果将包括 PH 相关生物标志物、右心室功能和患者报告结果的分析：本文为协议文章，结果将在数据收集完成后公布：POPLAR研究将提供数据，帮助更好地了解ILD-PH的病理生理学，改善PH和/或ILD患者的生活质量和预后：试验注册：日本临床试验注册中心：jRCT1010230018。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pulmonary Therapy Medicine-Pulmonary and Respiratory Medicine

CiteScore

5.20

自引率

3.30%

发文量

审稿时长

6 weeks

期刊介绍： Aims and Scope Pulmonary Therapy is an international, open access, peer-reviewed (single-blind), and rapid publication journal. The scope of the journal is broad and will consider all scientifically sound research from pre-clinical, clinical (all phases), observational, real-world, and health outcomes research around the use of pulmonary therapies, devices, and surgical techniques. Areas of focus include, but are not limited to: asthma; chronic obstructive pulmonary disease; idiopathic pulmonary fibrosis; pulmonary hypertension; cystic fibrosis; lung cancer; respiratory tract disorders; allergic rhinitis and other respiratory allergies; influenza, pneumococcal infection, respiratory syncytial virus and other respiratory infections; and inhalers and other device therapies. The journal is of interest to a broad audience of pharmaceutical and healthcare professionals and publishes original research, reviews, case reports/series, trial protocols and short communications such as commentaries and editorials. Pulmonary Therapy will consider all scientifically sound research be it positive, confirmatory or negative data. Submissions are welcomed whether they relate to an international and/or a country-specific audience, something that is crucially important when researchers are trying to target more specific patient populations. This inclusive approach allows the journal to assist in the dissemination of quality research, which may be considered of insufficient interest by other journals. Rapid Publication The journal’s publication timelines aim for a rapid peer review of 2 weeks. If an article is accepted it will be published 3–4 weeks from acceptance. The rapid timelines are achieved through the combination of a dedicated in-house editorial team, who manage article workflow, and an extensive Editorial and Advisory Board who assist with peer review. This allows the journal to support the rapid dissemination of research, whilst still providing robust peer review. Combined with the journal’s open access model this allows for the rapid, efficient communication of the latest research and reviews, fostering the advancement of pulmonary therapies. Open Access All articles published by Pulmonary Therapy are open access. Personal Service The journal’s dedicated in-house editorial team offer a personal “concierge service” meaning authors will always have an editorial contact able to update them on the status of their manuscript. The editorial team check all manuscripts to ensure that articles conform to the most recent COPE, GPP and ICMJE publishing guidelines. This supports the publication of ethically sound and transparent research. 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