Carvalho da Silva Marta Alexandra, Mouro de Santos Rolo Rui Miguel, Pereira Catarata Maria Joana, de Sousa Antunes Dias Padrão Eva Filipa
Pulmonology Department, Hospital de Braga, Braga, Portugal.
Breathe (Sheff). 2025 Apr 17;21(2):240176. doi: 10.1183/20734735.0176-2024. eCollection 2025 Apr.
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal interstitial fibrosing disease and, despite some well-known risk factors, its cause is still unknown. Cough is experienced by most patients and is commonly chronic and refractory, having a significant impact on quality of life. Its aetiology is complex, combining factors related to interstitial lung disease (ILD) such as an increased sensitivity of cough-sensitive nerves, structural lung changes and inflammation, genetic factors, several comorbidities and medication-adverse effects. Despite the therapeutic advancements in IPF over the past decade with the introduction of antifibrotic drugs that slow disease progression, effective treatment options for cough in IPF remain unavailable. Cough management often relies on empirical approaches based on studies involving chronic cough patients of unspecified causes and ILD physicians' personal experiences. Different classes of medications have been tried over time and, more recently, the focus has turned to neuromodulators and opioids, but several studies have shown suboptimal efficacy in cough. On the other hand, these drugs are associated with significant physical, psychological and economic burdens. However, the future brings us hope to the extent that most current ongoing clinical trials are using new molecules and some have demonstrated promising antitussive effects. This review aims to provide a practical guide to understanding and managing cough in IPF patients, presenting pharmacological and non-pharmacological approaches over time, as well as those treatments that are currently being investigated in clinical settings.
特发性肺纤维化（IPF）是一种慢性、进行性且致命的间质性纤维化疾病，尽管存在一些已知的危险因素，但其病因仍然不明。大多数患者会出现咳嗽，且通常为慢性难治性咳嗽，对生活质量有重大影响。其病因复杂，综合了与间质性肺疾病（ILD）相关的因素，如咳嗽敏感神经的敏感性增加、肺部结构改变和炎症、遗传因素、多种合并症以及药物不良反应。尽管在过去十年中，随着抗纤维化药物的引入，IPF的治疗取得了进展，这些药物可减缓疾病进展，但IPF咳嗽的有效治疗选择仍然缺乏。咳嗽管理通常依赖于基于对未明确病因的慢性咳嗽患者的研究以及ILD医生个人经验的经验性方法。随着时间的推移，人们尝试了不同类别的药物，最近，重点转向了神经调节剂和阿片类药物，但多项研究表明这些药物在治疗咳嗽方面效果欠佳。另一方面，这些药物会带来重大的身体、心理和经济负担。然而，未来给我们带来了希望，因为目前大多数正在进行的临床试验都在使用新分子，一些试验已显示出有前景的镇咳效果。本综述旨在为理解和管理IPF患者的咳嗽提供实用指南，介绍不同时期的药物和非药物方法，以及目前正在临床环境中研究的治疗方法。
