Abstract
The availability of epidemiological data relating to interstitial lung diseases (ILDs) has increased over recent years, but information on the prevalence and incidence of ILDs of different aetiologies remains limited. Despite global distribution, the proportion of patients who develop a progressive phenotype across different ILDs is not well known. Disease behaviour is well documented in idiopathic pulmonary fibrosis but idiosyncratic in other ILDs that may present a progressive fibrosing phenotype. Possible reasons may include the heterogeneous nature of the aetiology, the complexity of diagnosis (and subsequent documentation of cases) and the methods employed to retrospectively analyse patient databases. This review presents a broad overview of the epidemiological data available for ILDs that may present a progressive-fibrosing phenotype, collectively and stratified according to clinical classification. We also note where further data are needed in comparison to the well-studied IPF indication.
Abstract
ILDs with a progressive-fibrosing phenotype appear to be more common in older adults and are associated with a complex network of environmental and genetic factors. Further epidemiological studies are warranted to help identify these patients. http://ow.ly/SY6m30mWytM
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: A.L. Olson reports receiving the following, outside the submitted work: support from United Therapeutics for study enrolment IPF; support from Boehringer Ingelheim for unbranded IPF talks; support from France Foundation and the PILOT Program for project development for IPF/speaker/content development on talks for IPF; a grant from Bellerophon Pulse Technologies; and support from Boehringer Ingelheim Speaker's Bureau, Boehringer Ingelheim Consultancy, Genentech Advisory Board, Genentech Consultancy Services and Vindico CME Educational Development.
Conflict of interest: A.H. Gifford reports receiving nonfinancial support from Ashfield Healthcare Communications (part of UDG Healthcare pc) for assistance with preparation of the current work. A.H. Gifford also received personal fees from Grifols, outside the submitted work.
Conflict of interest: N. Inase has nothing to disclose.
Conflict of interest: E.R. Fernández Pérez reports receiving personal fees from BI, outside the submitted work.
Conflict of interest: T. Suda has nothing to disclose.
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 21, 2018.
- Copyright ©ERS 2018.
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