Introduction and Objectives Idiopathic pulmonary fibrosis (IPF) is associated with a reduced life expectancy of 2–3 years from diagnosis to death. In the CAPACITY¹ study patients treated with pirfenidone had a slower decline in FVC (evident from 6 months), and showed a trend towards improved mortality. We investigated causes of death and factors influencing mortality in patients who have received pirfenidone on a Named Patient Programme.

Method We retrospectively analysed the data from 25 patients with a minimum of 3 months follow-up who were treated with Pirfenidone between August 2011 and June 2012 in a tertiary ILD clinic.

Results Mean age was 67±8 years and 19 (76%) were male. 8 of the 25 patients treated died. 4 had discontinued pirfenidone prior to death. 4 died due to an IPF exacerbation, 3 due to progressive disease and 1 due to a combination of progressive disease and congestive cardiac failure. Survivors had a greater DLCO % predicted at baseline (49±16 v 30±8; p=0.001) and were heavier (82.8±14.6kg v 71.0±11.2kg; p=0.032). A greater proportion of subjects who died (75%) had severe IPF (DLCO<35% predicted or FVC<50% predicted) compared with those who survived (18%; p=0.014). For survivors compared with those who had died, there was a trend towards greater BMI (28.7±4.4 v 25.4±3.9; p=0.065) and younger age (65±8.2 v 70±4.5; p=0.053).

FVC % predicted, Chronic Respiratory Questionnaire Scores and the presence of significant co-morbidities at baseline did not predict survival.

Conclusion In IPF patients who had been treated with pirfenidone, all died of IPF related causes, and those who died weighed less, and had more severe disease with a lower baseline DLCO% predicted.

This may help to inform us about the most appropriate patients for whom pirfenidone treatment should be considered.

1. 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.