Abstract
There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation.
Abstract
Data for ILDs with a progressive fibrosing phenotype are lacking, but the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF http://ow.ly/Eoht30mS4Nx
Footnotes
Provenance: Publication of this peer-reviewed article was sponsored by Boehringer Ingelheim Pharmaceuticals Inc., Ridgefield, CT, USA (principal sponsor, European Respiratory Review issue 150).
Conflict of interest: C. Holtze reports non-financial support from Boehringer Ingelheim (writing assistance), during the conduct of the study.
Conflict of interest: K. Flaherty reports personal fees from Boehringer Ingelheim (consultancy – IPF 2016), Genentech (consultancy – IPF 2015), Veracyte (consultancy – IPF 2017), Roche (consultancy – IPF 2017), France Foundation (IPF CME content and presentations), Fibrogen (consultancy – IPF 2016) and Sanofi-Genzyme (consultancy – IPF 2017), outside the submitted work.
Conflict of interest: M. Kreuter reports grants and personal fees from Roche and Boehringer Ingelheim, during the conduct of the study.
Conflict of interest: F. Luppi reports personal fees from Boehringer Ingelheim, grants and personal fees from Roche, during the conduct of the study.
Conflict of interest: T. Moua has nothing to disclose.
Conflict of interest: C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work.
Conflict of interest: M.B. Scholand reports other from Boehringer Ingelheim (advisory board and investigator in clinical trials), Genentech (advisory board and investigator in a clinical trial), Fibrogen (investigator in a clinical trial), and Global Blood Therapeutics (investigator in a clinical trial), outside the submitted work. In addition, M.B. Scholand has a patent “Apparatus, compositions and methods for assessment of chronic obstructive pulmonary disease progression among rapid and slow decline conditions” issued.
Support statement: The authors received no direct compensation related to the development of the manuscript. Writing assistance was contracted and funded by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI).
- Received August 15, 2018.
- Accepted November 23, 2018.
- Copyright ©ERS 2018.
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