The Burden of Progressive-Fibrosing Interstitial Lung Diseases.

Despite conventional treatment, a proportion of interstitial lung disease (ILD) patients develop a progressive phenotype known as "fibrosing ILD with a progressive phenotype" (PF-ILD), characterized by worsening respiratory symptoms, decline in lung function, and early mortality. This review describes the epidemiology, and the humanistic and economic burden of PF-ILDs other than idiopathic pulmonary fibrosis (non-IPF PF-ILD). A structured review of the literature was conducted, using predefined search strategies in Ovid MEDLINE and EMBASE, and supplemented with gray literature searches.