Treatment patterns and patient journey in progressive pulmonary fibrosis: a cross-sectional survey.

IF 5.8 2区 医学 Q1 Medicine Respiratory Research Pub Date : 2024-10-09 DOI:10.1186/s12931-024-02995-9
Nazia Chaudhuri, Paolo Spagnolo, Claudia Valenzuela, Valeria C Amatto, Oliver-Thomas Carter, Lauren Lee, Mark Small, Michael Kreuter
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Abstract

Background: For patients with interstitial lung diseases (ILDs) presenting with a progressive pulmonary fibrosis (PPF) phenotype, current knowledge of disease characteristics at diagnosis, patient journey, and treatment is limited. This study aimed to describe demographics and clinical experiences of patients presenting with PPF in a European real-world setting.

Methods: Data were analysed from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey of pulmonologists and rheumatologists in five European countries (France, Germany, Italy, Spain, United Kingdom) and internal medicine specialists (France) from April to October 2022. Physicians provided data for up to 12 consecutive patients with physician-confirmed ILD with a progressive phenotype other than idiopathic pulmonary fibrosis. Analyses were descriptive.

Results: Overall, 265 physicians reported on 1,335 patients. Mean (standard deviation) age at survey date was 60.4 (11.6) years, 91.2% were white, 58.1% female, 44.0% non-smokers. Most patients (63.3%) first consulted a primary care physician. There was a mean delay of 7.8 (22.7) months between first ILD symptom and healthcare professional visit, and another 7.7 (12.8) months to ILD diagnosis. At survey date, 47.7% of patients had physician-reported moderate ILD, 42.3% had mild ILD and 10.0% had severe ILD. Disease progression was reported in the 12 months prior to the survey for 19.5% of patients; of these, progression was based on worsening symptom in 27.3% and lung function decline in 25.8%. For patients experiencing symptoms prior to ILD diagnosis (72.8%), the most common symptoms were dyspnoea on exertion (80.5%) and cough (57.8%). Overall, 17.4% of patients were misdiagnosed prior to ILD diagnosis, with chronic obstructive pulmonary disease suspected in 39.2% of them. The most frequent comorbidities were anxiety (16.9%) and gastroesophageal reflux (15.5%). Although 77.8% of patients were receiving treatment for ILD at survey date, 15.6% of patients had never been prescribed treatment for ILD.

Conclusions: This real-world study expands our understanding of patients, diagnostic delays and treatment gaps experienced by patients diagnosed with PPF in Europe. There was a mean delay of 15.5 months between first ILD symptoms and ILD diagnosis. Given the progressive nature of PPF, diagnostic delay may lead to poor outcomes, including shorter survival.

Trial registration: N/a.

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进行性肺纤维化的治疗模式和患者历程:横断面调查。
背景:对于表现为进行性肺纤维化(PPF)表型的间质性肺疾病(ILDs)患者,目前对其诊断时的疾病特征、患者历程和治疗方法的了解十分有限。本研究旨在描述欧洲真实世界中出现进行性肺纤维化的患者的人口统计学特征和临床经验:阿德尔菲真实世界 PPF-ILD 疾病专项计划™(Adelphi Real World PPF-ILD Disease Specific Programme™)于 2022 年 4 月至 10 月期间对欧洲五国(法国、德国、意大利、西班牙、英国)的肺病专家和风湿病专家以及内科专家(法国)进行了横断面调查,对调查数据进行了分析。医生们提供了最多 12 名经医生确诊的连续性 ILD 患者的数据,这些患者具有除特发性肺纤维化以外的进行性表型。分析为描述性分析:共有 265 名医生报告了 1,335 名患者的情况。调查时的平均年龄(标准差)为 60.4 (11.6) 岁,91.2% 为白人,58.1% 为女性,44.0% 不吸烟。大多数患者(63.3%)首先咨询的是初级保健医生。从首次出现 ILD 症状到就诊,平均延迟了 7.8 (22.7) 个月,到确诊 ILD 又延迟了 7.7 (12.8) 个月。在调查日期,47.7% 的患者由医生报告为中度 ILD,42.3% 为轻度 ILD,10.0% 为重度 ILD。据报告,19.5% 的患者在调查前的 12 个月内病情恶化;其中 27.3% 的患者病情恶化是因为症状恶化,25.8% 的患者是因为肺功能下降。在确诊 ILD 之前出现症状的患者(72.8%)中,最常见的症状是用力时呼吸困难(80.5%)和咳嗽(57.8%)。总体而言,有 17.4% 的患者在确诊 ILD 之前被误诊,其中 39.2% 的患者被怀疑患有慢性阻塞性肺病。最常见的合并症是焦虑(16.9%)和胃食管反流(15.5%)。尽管在调查日期,77.8% 的患者正在接受 ILD 治疗,但 15.6% 的患者从未接受过 ILD 治疗:这项真实世界的研究加深了我们对欧洲 PPF 患者、诊断延误和治疗差距的了解。从首次出现 ILD 症状到确诊 ILD,平均延迟了 15.5 个月。鉴于PPF的进展性,诊断延误可能导致不良后果,包括生存期缩短:未注册。
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来源期刊
Respiratory Research
Respiratory Research RESPIRATORY SYSTEM-
CiteScore
9.70
自引率
1.70%
发文量
314
审稿时长
4-8 weeks
期刊介绍: Respiratory Research publishes high-quality clinical and basic research, review and commentary articles on all aspects of respiratory medicine and related diseases. As the leading fully open access journal in the field, Respiratory Research provides an essential resource for pulmonologists, allergists, immunologists and other physicians, researchers, healthcare workers and medical students with worldwide dissemination of articles resulting in high visibility and generating international discussion. Topics of specific interest include asthma, chronic obstructive pulmonary disease, cystic fibrosis, genetics, infectious diseases, interstitial lung diseases, lung development, lung tumors, occupational and environmental factors, pulmonary circulation, pulmonary pharmacology and therapeutics, respiratory immunology, respiratory physiology, and sleep-related respiratory problems.
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