Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and fatal disease with an unknown cause. It is characterized by symptoms such as cough and breathlessness, which significantly impact patients’ quality of life. Cough, in particular, has emerged as a burdensome symptom for individuals with IPF. The etiology of cough in IPF patients is believed to be complex, involving factors related to the disease itself, such as increased sensitivity of cough nerves, lung structural changes, inflammation, and genetic factors, as well as comorbidities and medication effects. Unfortunately, effective treatment options for cough in IPF remain limited, often relying on empirical approaches based on studies involving chronic cough patients in general and the personal experience of physicians. Medications such as opioids and neuromodulators are commonly prescribed but have shown suboptimal efficacy, imposing significant physical, psychological, and economic burdens on patients. However, there is hope on the horizon, as specific purinergic P2 receptor ligand-gated ion channel (P2X3) inhibitors have demonstrated promising antitussive effects in ongoing clinical trials. This review aims to provide a comprehensive overview of the evaluation and management of cough in IPF patients, as well as highlight emerging pharmacological and non-pharmacological approaches that target the cough reflex and are currently being investigated in clinical settings.

特发性肺纤维化（IPF）是一种病因不明的慢性、进行性、致命性疾病。它的特点是咳嗽和呼吸困难等症状，严重影响患者的生活质量。尤其是咳嗽，已成为 IPF 患者的一种困扰症状。IPF患者咳嗽的病因被认为很复杂，涉及与疾病本身相关的因素，如咳嗽神经敏感性增加、肺部结构改变、炎症和遗传因素，以及合并症和药物作用等。不幸的是，针对 IPF 咳嗽的有效治疗选择仍然有限，通常依赖于基于对一般慢性咳嗽患者的研究和医生个人经验的经验方法。阿片类药物和神经调节剂等药物是常见的处方药物，但疗效不佳，给患者带来了显着的身体、心理和经济负担。然而，希望还是有的，因为特定的嘌呤能 P2 受体配体门控离子通道 (P2X3) 抑制剂在正在进行的临床试验中已证明具有良好的镇咳作用。本综述旨在全面概述 IPF 患者咳嗽的评估和治疗，并重点介绍针对咳嗽反射且目前正在临床环境中研究的新兴药理学和非药理学方法。