How many patients with idiopathic pulmonary fibrosis are there in Brazil?

Abstract

TO THE EDITOR: Idiopathic pulmonary fibrosis (IPF) is a form of chronic fibrosing interstitial pneumonia of unknown etiology that primarily affects the elderly and for which usual interstitial pneumonia is the substrate. (1) Patients with IPF usually experience progressive loss of pulmonary function and severely impaired quality of life, evolving to death. The treatment of IPF has always been a challenge. However, in late 2014, the US Food and Drug Admi-nistration approved two new drugs for the treatment of patients with this disease. Now it is clear that both drugs reduce the rate of decline in pulmonary function in individuals affected by the disease. In addition, clinical trials are underway to investigate new drugs with different mechanisms of action. We are seeing the beginning of a new era in the care of IPF patients, which is promising but also implies additional needs and concerns. In particular, health--related governmental decisions should be based on robust epidemiological data, and, unfortunately, there are few such data on IPF in Brazil. One important and still unresolved issue concerns the true incidence and prevalence of IPF and, consequently, the total number of affected patients in Brazil. When we analyzed the international literature, we found that characterizing the epidemiology of IPF is not a problem solely in Brazil. Some of the difficulties stem from the fact that the current definition of the disease came into use from 2000 onward. In addition, results vary depending on the criteria used by different authors to define a case of IPF. Nevertheless, there is a consensus that the disease affects more men than women, that it is more common after the fifth decade of life, and that its incidence has been increasing over the years, as has the associated mortality. (1)