Jastrzębski Dariusz, Kostorz-Nosal Sabina, Galle Dagmara, Gałeczka-Turkiewicz Alicja, Warzecha Joanna, Majewski Sebastian, Piotrowski Wojciech J, Ziora Dariusz
Department of Lung Diseases and Tuberculosis Faculty of Medical Sciences in Zabrze, Medical University of Silesia Zabrze Poland.
Stanisław Szyszko Independent Public Clinical Hospital No. 1 in Zabrze Medical University of Silesia Zabrze Poland.
Health Sci Rep. 2023 Aug 16;6(8):e1449. doi: 10.1002/hsr2.1449. eCollection 2023 Aug.
Antifibrotic therapies reduce lung function decline in patients with idiopathic pulmonary fibrosis (IPF). This single-arm, open-label, nonrandomized study aimed to determine the influence of antifibrotic treatment on patients' reported symptoms and expectations of the therapy.
Fifty-two patients with confirmed IPF at a mean age of 65 ± 8.63 years (73% male) completed the following surveys at baseline and after 12 months of Pirfenidone treatment: Short Form Healthy Survey (SF-36), St. George's Respiratory Questionnaire (SGRQ), Baseline Dyspnea Index (BDI), Fatigue Assessment Scale (FAS), Leicester Cough Questionnaire (LCQ), and Patient's Needs and Expectations Authors' Survey.
The most important patients' needs were access to novel therapy, fast and easy access to health centers specializing in IPF treatment, and the improvement of the general condition or the maintenance of its level. These needs did not change with time, except for the significantly more important right of deciding on disease management after 12 months of treatment ( = 0.014). The quality of life per SF-36, after 1 year of Pirfenidone treatment, significantly improved in the physical cumulative score ( = 0.004) and mental cumulative score ( = 0.003). Significant deteriorations were observed in bodily pain and vitality. For the remaining questionnaires (SGRQ, BDI, FAS, and LCQ), no significant changes in the course of the study were noticed. Around one in 10 patients subjected to Pirfenidone therapy had achieved general symptom improvement in all areas; that is, quality of life improvement as well as cough and dyspnea reduction.
One year of antifibrotic treatment resulted in a general improvement in the quality of life per the SF-36 questionnaire. Patients' expectations of disease management did not change; also, access to novel therapies and easy access to health centers specializing in IPF management remained their top needs.
抗纤维化疗法可减缓特发性肺纤维化（IPF）患者的肺功能衰退。本单臂、开放标签、非随机研究旨在确定抗纤维化治疗对患者报告症状及治疗期望的影响。
52例确诊为IPF的患者，平均年龄65±8.63岁（73%为男性），在基线期及接受吡非尼酮治疗12个月后完成以下调查：简明健康调查问卷（SF-36）、圣乔治呼吸问卷（SGRQ）、基线呼吸困难指数（BDI）、疲劳评估量表（FAS）、莱斯特咳嗽问卷（LCQ）以及患者需求与期望作者调查问卷。
患者最重要的需求是获得新疗法、快速便捷地前往专门治疗IPF的健康中心，以及改善总体状况或维持其水平。这些需求并未随时间变化，不过在治疗12个月后，疾病管理决策权变得更为重要（P = 0.014）。接受吡非尼酮治疗1年后，根据SF-36量表，身体累积评分（P = 0.004）和心理累积评分（P = 0.003）的生活质量显著改善。身体疼痛和活力方面出现显著恶化。对于其余问卷（SGRQ、BDI、FAS和LCQ），在研究过程中未观察到显著变化。接受吡非尼酮治疗的患者中，约十分之一在所有方面都实现了总体症状改善，即生活质量提高以及咳嗽和呼吸困难减轻。
抗纤维化治疗1年使SF-36问卷评估的生活质量总体得到改善。患者对疾病管理的期望未变；此外，获得新疗法以及便捷前往专门治疗IPF的健康中心仍是他们的首要需求。
