The burden of Progressive Fibrotic Interstitial lung disease across the UK

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]. Historically treatments for these cases have been limited though given the phenotypic similarities many cases may have been given a multidisciplinary working diagnosis of IPF based on their disease behaviour [3]. The INBUILD trial broadened the scope of treatable fILD by demonstrating a significant benefit of Nintedanib in patients with fILD and progressive disease [4]. In response to this the European Commission (EC) approved an additional indication for Nintedanib in adults for the treatment of PF-ILD in July 2020. Footnotes This manuscript has recently been accepted for publication in the European Respiratory Journal . It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article. Conflict of interest: Dr. Simpson reports non-financial support from Boehringer Ingelheim, outside the submitted work. Conflict of interest: Dr. Barratt reports personal fees from Boehringer Ingelheim, grants, personal fees and non-financial support from Boehringer Ingelheim, outside the submitted work. Conflict of interest: Dr. Beirne reports personal fees from Boehringer-Ingelheim Pharmaceuticals, non-financial support from Boehringer-Ingelheim Pharmaceuticals, outside the submitted work. Conflict of interest: Dr. Chaudhuri reports grants from Boehringer Ingelheim, personal fees from Boehringer Ingelheim, personal fees from Roche, outside the submitted work. Conflict of interest: Dr. Crawshaw reports I have received honoraria from Boehringer Ingelheim for educational meetings (chair/talks) and grant for virtual attendance at international conferences. Conflict of interest: Dr. Crowley has nothing to disclose. Conflict of interest: Dr. Fletcher has nothing to disclose. Conflict of interest: Dr. Gibbons reports that he has served on advisory boards and received support to attend conferences from Roche and Boehringer-Ingelheim. Conflict of interest: Dr. Hallchurch has nothing to disclose. Conflict of interest: Dr. Horgan has nothing to disclose. Conflict of interest: Dr. Jakaityte has nothing to disclose. Conflict of interest: Dr. Lewis has nothing to disclose. Conflict of interest: Dr. McLellan reports grants from Unrestricted Educational Grant (Boehringer Ingelheim), outside the submitted work. Conflict of interest: Dr. Myall has nothing to disclose. Conflict of interest: Dr. Miller has nothing to disclose. Conflict of interest: Dr. Smith has nothing to disclose. Conflict of interest: Dr. Stanel has nothing to disclose. Conflict of interest: Dr. Thillai has nothing to disclose. Conflict of interest: Dr. Thompson has nothing to disclose. Conflict of interest: Dr. Wallis has nothing to disclose. Conflict of interest: Dr. Wu has nothing to disclose. Conflict of interest: Dr. Molyneaux, via his institution received industry-academic funding from AstraZeneca and has received speaker and consultancy fees from Boehringer Ingelheim and Hoffman-La Roche outside the submitted work.Dr. Molyneaux reports personal fees from Boehringer Ingelheim, personal fees from Hoffman-La Roche, other from AstraZeneca, outside the submitted work. Conflict of interest: Dr. West has nothing to disclose.