Idiopathic pulmonary fibrosis: recent milestones in disease management

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia of unknown cause that is limited to the lungs [1]. The disease is inevitably fatal, with an estimated median survival of 2–5 yrs following diagnosis; the mortality rate in IPF is higher than mortality rates associated with a number of malignancies [2]. Historically, there were no pharmacological treatments approved in Europe for managing patients with IPF and there was a clear need for treatments, which may modify the disease course and help preserve lung function. This is now an exciting time for physicians, investigators and patients, as there is …