What is the treatment for cough in Interstitial Lung Disease (ILD)?

Treatment of Cough in Interstitial Lung Disease

For patients with ILD and refractory chronic cough, trials of therapies such as gabapentin, multimodality speech pathology therapy, and opiates for palliative care are recommended when alternative treatments have failed. 1

Initial Assessment and Management Algorithm

1. Rule out disease progression and complications

   • Assess for worsening of underlying ILD
   • Check for complications from immunosuppressive treatment (drug side effects, pulmonary infections)
   • Evaluate for common comorbidities causing cough 1

2. Investigate and treat common comorbidities

   • Gastroesophageal reflux disease (GERD) - particularly in scleroderma
   • Upper airway cough syndrome
   • Asthma/eosinophilic bronchitis
   • Note: Over 50% of ILD patients referred to cough clinics have cough caused by these common conditions 2

3. Disease-specific considerations

   • Idiopathic Pulmonary Fibrosis (IPF):

     • Antifibrotic therapy (pirfenidone, nintedanib) should be prescribed according to guidelines, not specifically for cough 1
     • Avoid proton pump inhibitors if negative workup for acid reflux 1

   • Sarcoidosis:

     • Inhaled corticosteroids should NOT be routinely prescribed (Grade 2C) 1

   • Scleroderma-associated ILD:

     • Cyclophosphamide and mycophenolate not supported solely for treating cough 1

Treatment for Refractory Cough in ILD

When cough persists despite addressing underlying causes and comorbidities:

1. First-line options for refractory cough:

   • Gabapentin - neuromodulator targeting cough hypersensitivity 1
   • Multimodality speech pathology therapy - cough suppression techniques 1

2. Second-line options:

   • Opiates (for severe, refractory cough affecting quality of life)
     • Recommended in palliative care setting
     • Reassess benefits/risks at 1 week and monthly thereafter
     • Consider increasing dose by approximately 20% in patients already receiving opioids for pain 1

Important Considerations and Caveats

• Chronic cough in ILD significantly impairs quality of life, comparable to unexplained chronic cough 1, 3
• Cough in IPF has prognostic significance, predicting disease progression independent of disease severity 1
• Cough mechanisms in ILD are complex, involving mechanical distortion, heightened cough reflex sensitivity, and airway inflammation 4, 3
• The evidence supporting management of chronic cough in ILD is limited, with most studies focusing on IPF 1
• Patients often report inadequate control of cough despite various medication trials, with only 18% reporting moderate benefit 5

Pitfalls to Avoid

• Don't automatically attribute cough to ILD - thoroughly evaluate for other common causes first 2
• Don't prescribe proton pump inhibitors for IPF patients with chronic cough and negative workup for acid reflux 1
• Don't routinely prescribe inhaled corticosteroids for cough in sarcoidosis 1
• Don't overlook non-pharmacological approaches - trigger avoidance is a common patient-reported strategy 5
• Don't delay palliative approaches when cough significantly impacts quality of life and is refractory to other treatments 1

The management of chronic cough in ILD requires a structured approach, first addressing disease progression and comorbidities, then moving to specific treatments for refractory cough, with gabapentin, speech therapy, and opiates (in palliative settings) being the mainstays of therapy when other approaches fail.