Idiopathic pulmonary fibrosis mortality: update on trends in the modern treatment era

Abstract

Extract

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease caused by a combination of genetic and environmental factors [1]. Studies indicate that the global incidence and prevalence of IPF are increasing [2–8]. The reasons for this are not fully understood, but contributors include more widespread use of computed tomography (CT) imaging, improved recognition of the disease, an ageing population, and greater exposure to environmental pollutants and other risk factors. Mortality rates in patients with well-defined IPF participating in multicentre clinical trials in the modern treatment era, which began in the early 2000s, have decreased (figure 1). Prior to that, the standard of care for IPF consisted of treatment with corticosteroids, azathioprine and other immunosuppressive medications, which have been shown to be associated with higher mortality [9–13].