What is the best approach for managing dry cough in pulmonary fibrosis?

Management of Dry Cough in Pulmonary Fibrosis

For patients with pulmonary fibrosis presenting with incapacitating dry cough, a transient, low-dose oral corticosteroid therapy should be prescribed when the cough is not alleviated by codeine, with careful monitoring of efficacy and tolerance. 1

Initial Assessment and Management Algorithm

• First, assess for progression of underlying pulmonary fibrosis or complications from immunosuppressive treatment (drug side effects, pulmonary infections) 1
• Evaluate for common comorbidities that may cause or worsen cough in pulmonary fibrosis patients:
  • Gastroesophageal reflux disease (GERD)
  • Upper airway cough syndrome
  • Obstructive sleep apnea syndrome 1, 2

First-Line Treatment Options

• Codeine should be tried as first-line therapy for dry cough in pulmonary fibrosis 1
• If codeine is ineffective, low-dose oral corticosteroids can be prescribed for a limited period 1
• Monitor both efficacy and tolerance of these medications closely 1

Important Considerations

• For patients with IPF and a negative workup for acid gastroesophageal reflux, proton pump inhibitor therapy should not be prescribed 1
• For patients with pulmonary sarcoidosis, inhaled corticosteroids should not be routinely prescribed to treat chronic cough 1
• High-dose corticosteroids or thalidomide are poorly tolerated and inadvisable for long-term management 1

Treatment for Refractory Cough

When first-line treatments fail and cough adversely affects quality of life:

• Opiates may be recommended for symptom control in a palliative care setting 1

  • Reassess benefits and risks at 1 week and then monthly before continuing
  • Low-dose morphine derivatives (<30 mg oral morphine equivalents daily) may be used with careful monitoring of side effects 1

• Consider therapies recommended for unexplained chronic cough:

  • Gabapentin or pregabalin (neuromodulators)
  • Multimodality speech pathology therapy 1

Monitoring and Follow-up

• Regular clinical visits and pulmonary function tests (including FVC) every 3-6 months 1
• Monitor for:
  • Progressive increase in dyspnea
  • Progressive decrease in FVC or DLCO
  • Worsening of signs on computed tomography 1

Pitfalls and Caveats

• Cough in pulmonary fibrosis is often multifactorial and may be due to the underlying disease, comorbidities, or both 2, 3
• Mechanical distortion from lung parenchymal fibrosis, heightened cough reflex sensitivity, and airway inflammation may all contribute to cough in IPF 2
• Cough is associated with poor prognosis in IPF and may precede dyspnea by years 2
• Treatments that work for unexplained chronic cough have not been extensively studied specifically in ILD-associated cough 1