吡非尼酮治疗特发性肺纤维化的真实世界疗效。
Real-world therapeutic performance of pirfenidone for connective tissue disease-associated interstitial lung diseases.
机构信息
Department of Rheumatology and Clinical Immunology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.
National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China.
出版信息
Ther Adv Respir Dis. 2024 Jan-Dec;18:17534666241292507. doi: 10.1177/17534666241292507.
BACKGROUND
Pirfenidone (PFD) is commonly applied for antifibrotic treatment in patients with idiopathic pulmonary fibrosis but has rarely been studied in cases with connective tissue disease-associated interstitial lung diseases (CTD-ILDs).
OBJECTIVES
We aimed to examine the efficacy of PFD in patients with CTD-ILD based on real-world data.
DESIGN
A retrospective cohort study.
METHODS
This study assessed the clinical features of CTD-ILD patients with or without a 6-month PFD treatment. A linear mixed effects model was employed to evaluate the effectiveness of PFD in alleviating lung function changes. Differences in response to PFD were analyzed based on CTD subtype, imaging classification, and pattern of pulmonary function at baseline.
RESULTS
A total of 289 patients with CTD-ILD were included, with 155 (53.6%) receiving PFD treatment and the remaining constituting the control group. Patients with the usual interstitial pneumonia (UIP) pattern were more likely to receive PFD treatment, and a relatively lower proportion of cases in the PFD group received immunosuppressive therapies compared to the control group ( < 0.05). At the 6-month follow-up, patients in the PFD group demonstrated a more significant improvement in forced vital capacity (FVC) and diffusion capacity for carbon monoxide (DLCO) (ΔFVC%: 2.9% vs 0.45%, = 0.009; ΔDLCO%: 1.9% vs -1.1%, = 0.004). In the linear mixed model analysis, there was a statistically significant group-time interaction between FVC% and DLCO% changes over time (FVC%: β = 4.52, < 0.001; DLCO%: β = 4.13, = 0.003). Furthermore, subgroup analysis indicated that pirfenidone may have superior therapeutic effects in patients with systemic sclerosis (SSc)-associated ILD, non-UIP pattern, and restrictive pattern of lung function at baseline.
CONCLUSION
This study provided real-world data demonstrating the effectiveness of PFD in terms of lung function improvement in patients with CTD-ILD.
背景
吡非尼酮（PFD）常用于治疗特发性肺纤维化患者的抗纤维化治疗，但在结缔组织病相关间质性肺病（CTD-ILD）患者中的研究甚少。
目的
我们旨在根据真实世界的数据研究 PFD 在 CTD-ILD 患者中的疗效。
设计
回顾性队列研究。
方法
本研究评估了有或无 6 个月 PFD 治疗的 CTD-ILD 患者的临床特征。采用线性混合效应模型评估 PFD 缓解肺功能变化的疗效。根据 CTD 亚型、影像学分类和基线时的肺功能模式分析对 PFD 反应的差异。
结果
共纳入 289 例 CTD-ILD 患者，其中 155 例（53.6%）接受 PFD 治疗，其余为对照组。普通间质性肺炎（UIP）模式的患者更有可能接受 PFD 治疗，且 PFD 组接受免疫抑制治疗的比例相对低于对照组（<0.05）。在 6 个月随访时，PFD 组患者用力肺活量（FVC）和一氧化碳弥散量（DLCO）改善更明显（ΔFVC%：2.9%比 0.45%，=0.009；ΔDLCO%：1.9%比-1.1%，=0.004）。在线性混合模型分析中，FVC%和 DLCO%随时间的变化之间存在统计学显著的组-时间交互作用（FVC%：β=4.52，<0.001；DLCO%：β=4.13，=0.003）。此外，亚组分析表明，吡非尼酮在系统性硬化症（SSc）相关间质性肺病、非 UIP 模式和基线时限制性肺功能模式的患者中可能具有更好的治疗效果。
结论
本研究提供了真实世界的数据，表明 PFD 可改善 CTD-ILD 患者的肺功能。
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