Progressive Fibrotic Interstitial Lung Disease
摘要
The term progressive fibrotic interstitial lung disease (PF-ILD) describes patients with fibrotic ILD who, at some point in time, exhibit a progressive phenotype. The terminology is independent of the specific label of the prevailing ILD. Idiopathic pulmonary fibrosis (IPF) by its very nature is progressive, but many other ILDs are not necessarily so. This chapter addresses the issues in the diagnostic labelling of PF-ILD, the factors that need to be sought in a patient with ILD progression, and the relevance of progressive disease in patients within each subset of ILD.