Abstract
Almost 15% of new referrals with non-IPF fibrotic ILD go on to develop a progressive fibrotic phenotype and would benefit from antifibrotic therapy https://bit.ly/3uPhClN
Highlights
While idiopathic pulmonary fibrosis (IPF) remains the exemplar progressive fibrotic lung disease, there remains a cohort of non-IPF fibrotic lung diseases which adopt a similar clinical behaviour to IPF despite therapy [1]
This phenotypically related group of conditions, where progression of disease is similar to that seen in IPF, have recently been described as progressive fibrotic interstitial lung diseases (PF-ILD) [2]
All new referrals seen for their first outpatient clinic appointment between August 1, 2017, and January 31, 2018, were assessed against the diagnostic criteria for PF-ILD laid out in the INBUILD trial [8] and, in particular, the criteria for progression: relative decline in forced vital capacity (FVC) % predicted ⩾10%, or FVC decline ⩾5% but
Summary
While idiopathic pulmonary fibrosis (IPF) remains the exemplar progressive fibrotic lung disease, there remains a cohort of non-IPF fibrotic lung diseases (fILD) which adopt a similar clinical behaviour to IPF despite therapy [1] This phenotypically related group of conditions, where progression of disease is similar to that seen in IPF, have recently been described as progressive fibrotic interstitial lung diseases (PF-ILD) [2]. In the cohort of patients at risk of developing PF-ILD the INBUILD criteria were met in 14.5% (253 out of 1749) of all new non-IPF fILD referrals despite standard therapy, with a range between these specialist ILD centres from 8.9% to 23.6% of total cases.
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