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Assessment and Management of Cough in Idiopathic Pulmonary Fibrosis: A Narrative Review

  • REVIEW: COUGH IN IDIOPATHIC PULMONARY FIBROSIS
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Abstract

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.

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Abbreviations

ATP:

Adenosine triphosphate

ABCs:

Airway basal cells

ACEIs:

Angiotensin-converting enzyme inhibitors

ARB:

Angiotensin receptor blockers

BALF:

Bronchoalveolar lavage fluid

BDNF:

Brain-derived neurotrophic factor

CPAP:

Continuous positive airway pressure

CVA:

Cough variant asthma

FVC:

Forced vital capacity

GERD:

Gastroesophageal reflux disease

IPF:

Idiopathic pulmonary fibrosis

ILD:

Interstitial lung disease

LCQ:

Leicester cough questionnaire

NGF:

Nerve growth factor

NAEB:

Non-asthmatic eosinophilic bronchitis

NAL-ER:

Nalbuphine-extended release

OSAS:

Obstructive sleep apnea syndrome

UACS:

Upper airway cough syndrome

VAS:

Visual analogue scale

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Authors and Affiliations

  1. Department of Intensive Care Medicine, Nanjing Jiangbei Hospital, Nanjing, China

    Shangxiang Liu

  2. Department of Respiratory and Critical Care Medicine, Nanjing Drum Tower Hospital, Affiliated Hospital of Medical School, Nanjing University, No. 321 Zhongshan Road, Nanjing, China

    Xu Ye

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  1. Shangxiang Liu
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  2. Xu Ye
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SL wrote the main manuscript text and prepared Fig. 1 and Tables 1–2. XY was responsible for revising and reviewing the manuscript and finalize the manuscript. All authors reviewed the manuscript.

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Correspondence to Xu Ye.

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Liu, S., Ye, X. Assessment and Management of Cough in Idiopathic Pulmonary Fibrosis: A Narrative Review. Lung 201, 531–544 (2023). https://doi.org/10.1007/s00408-023-00653-3

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