Pirfenidone Improves Objective and Subjective Cough Measures in Patients with Idiopathic Pulmonary Fibrosis

Introduction

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal lung disease. Cough, being one of the most disabling symptoms, independently predicts the disease progression. However, there are no effective treatments for cough in IPF. Pirfenidone, an anti-fibrotic drug, might be effective in reducing cough according to several observations.  

Aim

This study evaluates the impact of pirfenidone on objective and subjective measures of cough in patients with IPF. The effect of pirfenidone on the quality of life (QoL) is also assessed.

Method

Study Design

• International, multicentre, prospective, observational study
• The cohort included treatment-na?ve IPF patients aged 40–85?years with a forced vital capacity (FVC) ≥50% and corrected transfer factor of the lung for carbon monoxide (T_LCOc) ≥30%
• Pirfenidone therapy was about to be initiated in these patients
• Patients had IPF-related cough for ≥8?weeks with a cough score of ≥40?mm on a 0–100?mm visual analogue scale (VAS)

Treatment Strategy

• 24-hr cough recording was done at baseline, and after 4 and 12 weeks of therapy with the Leicester Cough Monitor (LCM)
• The patients filled in the Leicester Cough Questionnaire (LCQ), the VAS cough severity, VAS urge-to-cough, King's Brief Interstitial Lung Disease health status questionnaire (K-BILD), the Hospital Anxiety and Depression Scale (HADS) and the seven-item Generalised Anxiety Disorder screener (GAD-7) at baseline and after 4 and 12 weeks of therapy

End-points

• Objective cough frequency
• Subjective cough measures

Results

• Out of 43 patients, 38 patients completed 4 weeks of the study, and 31 completed 12 weeks
• Objective 24-hr cough reduced by 34% after 12 weeks of pirfenidone therapy
• 74% reported an improvement in 24-hr cough
• There were significant improvements in the subjective cough measures as shown in table 1.

• There were no significant changes in disease-specific QoL and anxiety
• At 4 weeks of therapy, there was a 14% reduction in 24-hr cough frequency
• 69% reported improvements in cough at 4 weeks

Conclusions

• Treatment with pirfenidone resulted in significant improvements in objective 24-hr cough counts and subjective measures of cough in patients with idiopathic pulmonary fibrosis (IPF).
• These findings are of clinical significance to the IPF patients.

Eur Respir J. 2017 Oct; 50(4): 1701157. Doi: 10.1183/13993003.01157-2017.