LAUSR

In this issue, the European Respiratory Review publishes a series of articles on fibrosing interstitial lung diseases (ILDs) that present with a progressive phenotype. Progressive-fibrosing ILD (PF-ILD) is a terminology recently used to describe these patients. In addition to patients with idiopathic pulmonary fibrosis (IPF), there is a large and more diverse group of patients who develop PF-ILD. These patients are suffering from a disorder that shares a number of clinical features with IPF including: increasing respiratory symptoms; decline of the physiological functions; limited response to immunomodulatory therapy; premature mortality; and significantly impaired quality of life. These articles raise the awareness of these PF-ILDs and review our current understanding of their clinical behaviour and management. Olson et al. [1] report on the present status of epidemiology, with an overview on the incidence and prevalence of progressive-fibrosing lung disease. The presentation, diagnosis and clinical course of the spectrum of PF-ILD is well described by Cottin et al. [2], while the role of imaging is analysed in depth by Walsh et al. [3]. A detailed analysis of the key role, burden, management and definition of acute exacerbations in PF-ILD is provided by Kolb et al. [4]. Finally, Richeldi et al. [5] provide an interesting overview on the currently available management options in PF-ILD including possible future alternatives, while Swigris et al. [6] present a review on the evaluation and monitoring of the currently available patient-reported outcome instruments and Holtze et al. [7] discusses the problems related to the healthcare burden of these diseases. Beyond IPF: the world of PF-ILD http://ow.ly/zZZ430mYeaE