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

摘要

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]. In addition to IPF, a number of fibrosing ILDs can develop a progressive phenotype characterised histologically by self-sustaining fibrosis, a process common to a variety of conditions, and which leads to worsening quality of life, decline in lung function and, eventually, early mortality. Because these conditions share similarities regarding pathogenesis and clinical behaviour, they are increasingly described under the umbrella terminology of “progressive fibrosing ILDs” (PF-ILDs) or “fibrosing ILD with a progressive phenotype” [3, 4]. In this editorial, PF-ILDs will refer to fibrosing ILDs other than IPF which have a progressive phenotype.