Well-validated relevant PROMs can be employed for multiple purposes: as outcome measures for daily care or for driving therapeutic decisions, as efficacy endpoints in clinical trials, or as tools to collect useful data for healthcare policy makers in order to improve access and quality of care.

Idiopathic pulmonary fibrosis (IPF) is a progressive, fatal lung disease. In patients with IPF, cough is one of the most disabling symptoms and is an independent predictor of disease progression [1][2][3]. Cough in IPF is often non-responsive to antitussive therapy [4], and studies on cough are scarce and have unfortunately not yet resulted in effective treatments. Several observations suggest that pirfenidone, an anti-fibrotic drug, might decrease cough [5, 6]. We aimed to measure objectively the effect of pirfenidone on cough in patients with IPF and substantial cough. In addition, we assessed the effect of pirfenidone on subjective cough and quality of life (QoL) measures.This international, multicentre, prospective, observational study at four sites (The Netherlands, Italy, France and UK) recruited patients between 2013 and 2016. Treatment-naïve IPF patients aged 40-85 years with a forced vital capacity (FVC) ⩾50% and corrected transfer factor of the lung for carbon monoxide (TLCOc) ⩾30%, in whom pirfenidone therapy was about to be initiated according to regular practice, who had daily IPF-related cough for ⩾8 weeks with a cough score of ⩾40 mm on a 0-100 mm visual analogue scale (VAS), were eligible for the present study.Patients underwent baseline 24-h cough recording with the Leicester Cough Monitor (LCM) prior to starting pirfenidone, and after 4 and 12 weeks of treatment. The LCM is a validated ambulatory cough monitoring system [7]. Recordings were centrally analysed with automated cough software as previously described [7]. On the days of cough recording, participants completed the Leicester Cough Questionnaire (LCQ, a cough-specific health status questionnaire), 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) [8,9].Analyses were performed on the intention-to-treat population. Objective cough frequency data were log transformed. To assess the effect of pirfenidone on cough over time we used a linear mixed model, which accounts for missing data [10]. Sensitivity analyses were performed on the per protocol population with a linear mixed model and paired t-tests. SPSS Statistics version 21.0 and R version 3.2.2 software packages were used for statistical analysis. The study was approved by the ethics committees of all participating centres (clinical trials.gov, NCT02009293). All patients provided written informed consent.Of the 46 patients screened for the study, 43 patients were included. Reasons for exclusion were low TLCOc and/or VAS scores. 38 patients completed 4 weeks of the study, and 31 completed 12 weeks. Reasons for dropout included: cessation of pirfenidone because of adverse events (8), logistic reasons (2), change of proton pomp inhibitor (1) and death (1). The age of the patients was 72±7 (mean±SD); most were men (33, 77%) and former smokers (34, 79%). Baseline cough counts, questionnaire scores and pulmonar...

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