The increasing mortality of idiopathic pulmonary fibrosis: fact or fallacy?

There is no debate that idiopathic pulmonary fibrosis (IPF) is a devastating disease that has a significant impact on patient morbidity and mortality. Patients with IPF experience symptoms throughout most of their disease, with disabling dyspnoea and cough that reduce quality of life. Most IPF patients eventually succumb to respiratory failure with a median survival of approximately 3 years from the time of diagnosis [1]. Two antifibrotic medications slow progression of IPF [2–5]; however, it has yet to be shown whether IPF mortality will change substantially with the availability of these therapies. There are likely multiple reasons that IPF-related mortality appears to be increasing in most European countries