Idiopathic Pulmonary Fibrosis (IPF): Should it be all about the FVC?

Boot S Horgan L Flint L Sutherland T Beirne P Leeds Teaching Hospitals NHS Trust Antifibrotic therapy in the UK is restricted to patients with Forced Vital Capacity (FVC) 50-80%. Richeldi showed in trial data the rate of decline in IPF patients whose FVC is above or below 80% to be comparable. We studied 163 patients with a MDT diagnosis of IPF before February 2016 who were eligible for antifibrotic treatment by the guidelines and compared those to a random selection of patients above treatment parameters. In a Specialist Centre we compared those with FVC>80% to those FVC<80% using the GAP (Gender, Age, Physiology) score, Pack Year History (PYH), Diffusing Capacity for Carbon Monoxide (DLCO) and mortality. PYH was comparable in both, suggesting smoking history does not explain FVC preservation. FVC is an independent mortality predictor, but despite this nearly half of the >80% group died by the end of the study period. Our clinical data supports earlier trial work that IPF has the same trajectory of decline, regardless of initial FVC, and is inevitably fatal. FVC is currently the only eligibility criterion in antifibrotic therapy in the UK; this merits review to prevent discrimination against those with a higher initial FVC. Richeldi L, Cottin V, du Bois M, Selman M, Kimura T, Bailes Z & Brown K (2016) Nintedanib in patients with idiopathic pulmonary fibrosis:Combined evidence from TOMORROW and INPULSIS® trials. Resp Med, 113, 74-79