Abstract
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.