Time for Prevention of Idiopathic Pulmonary Fibrosis Exacerbation.

Abstract

Acute exacerbations of idiopathic pulmonary fibrosis (AE-IPF), with an annual incidence up to 20% and a short-term mortality around 50%, are the more frequent cause of death in patients with IPF. These unpredictable and clinically relevant events are more frequent in patients with advanced disease, as measured by a lower lung function. In the absence of an evidence-based, approved treatment of AE-IPF, patients with IPF with acute respiratory worsening are usually treated with systemic high-dose corticosteroids according to current guideline recommendations. Part of the reason for the lack of a treatment for AE-IPF with proven safety and efficacy is the difficulty in designing clinical trials addressing this specific clinical endpoint. Another part of the problem is represented by the current definition of AE-IPF, until now based on clinical and radiological features, and the absence of an identifiable etiology. Because a number of studies have shown that the features and prognosis of AE-IPF are similar to other causes of acute respiratory worsening, recently a change in the definition of AE-IPF has been proposed, focusing more on clinical and radiological findings consistent with an underlying pathobiology of diffuse alveolar damage and placing less emphasis on the search for the etiological cause. It is hoped that the international scientific community will soon be able to reach a consensus on a new definition of AE-IPF, thus speeding up research for an effective and safe treatment.