Article Text
Abstract
Introduction and Objectives Pirfenidone is currently the only disease-modifying therapy for idiopathic pulmonary fibrosis. However the CAPACITY1 study demonstrated frequent mild adverse effects. No data is available on the overall effect of pirfenidone on quality of life. Our aim was to describe patients’ experience of pirfenidone, taking these factors in to account.
Methods This was a single-centre observational study of patients participating in the pirfenidone named patient programme. Participants completed the Self-Report Chronic Respiratory Disease Questionnaire (SR-CRQ), a quality of life assessment with four domains, at three monthly intervals. Adverse effects were also recorded. Patients were followed up until death or cessation of pirfenidone.
Results 26 patients were included, with 9 patients reaching 3 months, 6 reaching 6 months, and 5 reaching 9 months during the study period. Mean age was 67±7 years, and 77% were male. There was a downward trend in all domains of the SR-CRQ, as illustrated in figure 1. However there were no significant differences in scores from baseline compared with individual time points. No relationship was found between SR-CRQ scores and adverse effects.
Adverse effects were experienced by 14 patients (54%). These included gastrointestinal symptoms (n=12), photosensitivity (n=2) and fatigue (n=3). 5 patients stopped pirfenidone as a result of adverse effects. The mean weight in kilogrames of those patients experiencing adverse effects was lower than those who did not (72.5±12.7 vs 86.1±13.2, p=0.014). Age in years was higher in those with adverse effects (70±6 vs 64±8, p=0.035). Patients with more severe disease (FVC<50%, DLCO <35%) were also significantly more likely to have adverse effects (p=0.005).
Conclusion The data from this study is limited due to small numbers, however we have not observed a significant change in quality of life from baseline in those treated with pirfenidone. Adverse effects were greater in older, lighter patients with more severe disease.
Noble P, Albera C, Bradford W et al, for the CAPACITY Study Group. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet 2011; 377: 1760–69.