A first step against idiopathic pulmonary fibrosis

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic devastating disease affecting 50 to 300 individuals per million with a median survival time of ∼3 yrs [1]. The recent publication of evidence-based guidelines for the diagnosis and management of patients with IPF did not provide patients and care givers with any reason for optimism [2]. The only recommended procedures in this document were: long-term oxygen therapy in patients with clinically significant hypoxaemia; lung transplantation in selected patients; treatment of asymptomatic gastro-oesophageal reflux (although there is not even one randomised trial showing that this treatment improves lung function decline or survival in IPF patients); pulmonary rehabilitation; and corticosteroid use in patients with acute exacerbation of IPF [3]. Almost all treatment procedures evaluated in that statement were given either a weak or a strong recommendation against use [2]. However, the litany of the negative therapeutic trials in IPF ended with the recent publication of the results of three phase III trials evaluating the efficacy of pirfenidone [4–6]. The results of these trials, including >1,000 …