What is the treatment for refractory cough in Idiopathic Pulmonary Fibrosis (IPF)?

Treatment of Refractory Cough in Idiopathic Pulmonary Fibrosis (IPF)

For patients with refractory cough in IPF, gabapentin, multimodality speech pathology therapy, and opiates (particularly low-dose controlled-release morphine) are recommended treatment options when other therapies have failed. 1, 2

Initial Assessment and Management

• First, assess for progression of underlying IPF or complications from immunosuppressive treatment (e.g., drug side effects, pulmonary infection) before initiating specific cough treatments 1
• Evaluate for common comorbidities that may contribute to cough, including gastroesophageal reflux disease (GERD), obstructive sleep apnea, and pulmonary hypertension 1, 3
• If GERD workup is negative, proton pump inhibitor therapy should not be prescribed for cough management 1

Treatment Algorithm for Refractory Cough in IPF

First-line Options:

• Gabapentin therapy: Follow dosing protocols as recommended for unexplained chronic cough 1
• Multimodality speech pathology therapy: Includes cough suppression techniques and breathing exercises 1

Second-line Option:

• Low-dose controlled-release morphine (5 mg twice daily): Recent high-quality evidence shows 39.4% reduction in objective awake cough frequency compared to placebo 2
  • Monitor for common side effects: constipation (21%) and nausea (14%) 2
  • Reassess benefits and risks at 1 week and then monthly before continuing 1

Evidence Quality and Considerations

• The 2018 CHEST guidelines provide consensus-based recommendations due to limited high-quality evidence specifically for IPF-related cough 1
• A recent (2024) randomized controlled trial demonstrated significant efficacy of low-dose controlled-release morphine, making it a particularly strong option based on the latest evidence 2
• Thalidomide showed promise in a small trial for improving cough-specific quality of life in IPF patients, but this recommendation did not pass the CHEST guideline panel vote 1, 4

Important Caveats and Pitfalls

• Cough in IPF has prognostic significance and is associated with disease progression, independent of disease severity 1
• Up to 80% of IPF patients experience chronic cough, which significantly impairs quality of life 1, 5
• The pathophysiology of cough in IPF is multifactorial, involving mechanical distortion from fibrosis, heightened cough reflex sensitivity, and possibly airway inflammation 1
• Pirfenidone is approved for IPF treatment and may help slow disease progression, but it is not specifically indicated for cough management 6, 7
• Clinical trials for cough treatment in IPF are emerging, so consider referring patients to available clinical trials 1, 5

Palliative Considerations

• For patients with chronic cough due to IPF that adversely affects quality of life and is refractory to other treatments, opiates should be considered in a palliative care setting 1
• Regular reassessment of benefits versus risks is essential when using opiates for symptom control 1