Yasuoka Hidekata, Waseda Yuko, Kaneko Yuko, Okazaki Masateru, Iwasaki Ryoko, Nagata Shoko, Small Mark, Ishii Haruyuki
Division of Rheumatology, Department of Internal Medicine, School of Medicine, Fujita Health University, Toyoake, Japan.
Department of Respiratory Medicine, Faculty of Medical Sciences, University of Fukui, Eiheiji, Japan.
Front Med (Lausanne). 2025 Jan 15;11:1526531. doi: 10.3389/fmed.2024.1526531. eCollection 2024.
There is a paucity of real-world data on patients with interstitial lung diseases (ILDs) that are progressive, other than idiopathic pulmonary fibrosis (IPF), including treatment patterns and attitudes toward treatment. This study aimed to investigate the diagnosis, clinical characteristics, treatment paradigm and current decision-making practices of IPF and progressive pulmonary fibrosis (PPF) in a Japanese real-world setting.
Data were drawn from the Adelphi Real World PPF-ILD Disease Specific Programme™, a cross-sectional survey with retrospective data collection of pulmonologists and rheumatologists in Japan from April to October 2022. Physicians provided data for up to 12 consecutive patients with a physician-confirmed diagnosis of progressive ILD; patients were also invited to complete patient self-completion forms. Analyses were descriptive.
A total of 63 physicians (43 pulmonologists and 20 rheumatologists) provided data on 312 patients with PPF and 70 patients with IPF. Patients had a mean (standard deviation [SD]) age at survey date of 68.0 (11.6) years, 43.5% were female, 50.3% were former smokers and 18.1% were employed full time. For breathlessness, 26.5% of patients had Grade 2 physician-reported breathlessness; this was 16.7% when reported by patients themselves. A total of 81.4% of patients were currently receiving treatment for ILD. Mean (SD) duration of current treatment was 1.5 (1.4) years. Slowing disease progression was the primary reason influencing physicians' choice of current ILD treatment (48.5%). A total of 16.0% had never been treated (most frequent physician-reported reason: disease was manageable without treatment, 55.7%) and 2.6% had treatment discontinued (most frequent reason: patient request, 70.0%). Physicians reported 82.3% of patients as fully compliant with their treatment regimen. As reported by patients themselves ( = 53), 49.1% never and 37.7% rarely missed a dose.
This analysis of real-world data from Japan provides insights into the clinical profile of patients with IPF and PPF in Japan, and highlights differences between physicians and patients in perception of symptom severity and attitudes to treatment.
除特发性肺纤维化（IPF）外，关于进行性间质性肺疾病（ILDs）患者的真实世界数据匮乏，包括治疗模式和对治疗的态度。本研究旨在调查日本真实世界环境中IPF和进行性肺纤维化（PPF）的诊断、临床特征、治疗模式及当前的决策实践。
数据来自阿德尔菲真实世界PPF-ILD疾病特定项目™，这是一项横断面调查，于2022年4月至10月收集了日本肺科医生和风湿科医生的回顾性数据。医生提供了多达12例经医生确诊为进行性ILD的连续患者的数据；患者也被邀请填写患者自填表格。分析为描述性分析。
共有63名医生（43名肺科医生和20名风湿科医生）提供了312例PPF患者和70例IPF患者的数据。调查时患者的平均（标准差[SD]）年龄为68.0（11.6）岁，43.5%为女性，50.3%为既往吸烟者，18.1%为全职工作。对于呼吸困难，26.5%的患者有医生报告的2级呼吸困难；患者自己报告的这一比例为16.7%。共有81.4%的患者目前正在接受ILD治疗。当前治疗的平均（SD）持续时间为1.5（1.4）年。减缓疾病进展是影响医生选择当前ILD治疗的主要原因（48.5%）。共有16.0%的患者从未接受过治疗（医生报告的最常见原因：疾病无需治疗即可控制，55.7%），2.6%的患者治疗中断（最常见原因：患者要求，70.0%）。医生报告82.3%的患者完全遵守治疗方案。据患者自己报告（n = 53），49.1%的患者从未漏服过药物，37.7%的患者很少漏服。
这项对日本真实世界数据的分析提供了对日本IPF和PPF患者临床特征的见解，并突出了医生和患者在症状严重程度认知和治疗态度上的差异。
