日本肺动脉高压患者的诊断延迟和生活质量：一项全国性调查。

IF 2.3 Q2 RESPIRATORY SYSTEM

Pulmonary Therapy Pub Date : 2025-06-01 Epub Date: 2025-03-18 DOI:10.1007/s41030-025-00290-6

Noriko Murakami, Daiki Asano, Natsuko Tokushige, Junichi Omura, Megumi Watanabe, Seitaro Nomura, Hiroaki Kitaoka, Yuichi Tamura

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引用次数: 0

摘要

肺动脉高压（Pulmonary hypertension， PH）是一种罕见且严重的疾病，严重影响患者的生活。然而，日本对这种疾病的诊断和治疗的全面情况仍不清楚。本研究旨在通过一项针对PH患者和治疗医生的全国性调查来阐明这些方面。方法：对160例PH患者（119例合并肺动脉高压[PAH]， 41例合并慢性血栓栓塞性肺动脉高压[CTEPH]）进行横断面调查，其中121例为女性（75.6%），来自日本的211名医生。问卷评估了患者的诊断过程、就业状况、与医生关于治疗目标的沟通、健康相关生活质量（HRQoL）和药物依从性。结果：患者在接受PH诊断前平均访问了2.3家医疗机构(PAH患者：2.2家；CTEPH患者：2.3次就诊)，从症状出现到诊断平均时间为18.0个月(PAH: 20.2个月；CTEPH: 12.2个月)。就业率和入学率从诊断前的68.8%下降到诊断后的44.4%，进一步下降到调查时的36.9%。患者和医生之间关于治疗目标的沟通存在差异，特别是在患有CTEPH的患者中（82.9%的患者报告了这种讨论，而治疗医生的这一比例为41.2%）。通过强调-10问卷评估的HRQoL得分中位数表明存在损害(PAH: 21.5；CTEPH: 18.0)，随着疾病严重程度的增加而恶化。结论：这项全国性的研究提供了日本PH患者面临的挑战的全面概述。研究结果表明，早期诊断，支持患者就业和教育，改善医患沟通，以减轻PH负担，提高患者预后是必不可少的。本文提供图形摘要。

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Diagnostic Delays and Quality of Life in Japanese Patients with Pulmonary Hypertension: A Nationwide Survey.

Introduction: Pulmonary hypertension (PH) is a rare and severe disorder that significantly affects patients' lives. However, a comprehensive picture of the diagnosis and treatment of this condition in Japan remains unclear. This study aimed to elucidate these aspects by conducting a nationwide survey targeting patients with PH and treating physicians.

Methods: A cross-sectional survey was conducted among 160 patients with PH (119 with pulmonary arterial hypertension [PAH] and 41 with chronic thromboembolic pulmonary hypertension [CTEPH]), of whom 121 were female (75.6%), and 211 physicians across Japan. The questionnaires assessed patients' diagnostic journey, employment status, communication with physicians regarding treatment goals, health-related quality of life (HRQoL), and medication adherence.

Results: Patients visited a mean of 2.3 medical facilities before receiving a PH diagnosis (PAH patients: 2.2 visits; CTEPH patients: 2.3 visits), with a mean time from symptom onset to diagnosis of 18.0 months (PAH: 20.2 months; CTEPH: 12.2 months). Employment and school attendance rates declined from 68.8% before diagnosis to 44.4% immediately after diagnosis, and further to 36.9% at the time of the survey. Discrepancies in communication about treatment goals were observed between patients and physicians, particularly in patients with CTEPH (82.9% of patients reported such discussions vs. 41.2% of treating physicians). Median HRQoL scores, as assessed by the emPHasis-10 questionnaire, indicated impairment (PAH: 21.5; CTEPH: 18.0), which worsened with increasing disease severity.

Conclusion: This nationwide study provides a comprehensive overview of the challenges faced by patients with PH in Japan. The findings suggest the essential need for earlier diagnosis, support for employment and education among patients, and improved patient-physician communication to reduce the burden of PH and enhance patient outcomes. Graphical abstract avaliable for this article.

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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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