Treatment of progressive fibrosing interstitial lung diseases: a milestone in the management of interstitial lung diseases

Interstitial lung disease (ILD) refers to a large and heterogeneous group of parenchymal lung disorders [1], some related to other diseases such as connective tissue diseases (CTDs), some related to environmental exposures such as hypersensitivity pneumonitis, and others with an unknown cause and no identified aetiologic context. Idiopathic pulmonary fibrosis (IPF) is the most common type of idiopathic ILD, the most severe of the chronic forms of ILDs and represents, by definition, the prototype of progressive fibrosing ILD characterised by decline in lung function and early mortality [2]. Fibrosing ILDs can develop a progressive phenotype and are described under the terminology PF-ILDs. Due to commonalities with IPF, the potential efficacy and tolerability of antifibrotic drugs pirfenidone and nintedanib are currently evaluated in PF-ILDs. http://bit.ly/2koL0A4