Abstract
The additional argument advanced by Professor Cottin for retention of the term “idiopathic pulmonary fibrosis” is highly persuasive http://ow.ly/n05630nVCZd
From the authors:
The writer makes a powerful plea for clarity of thought based on clarity of language, arguing from the Socratic maxim that “the beginning of wisdom is the definition of terms”. This etymological perspective is extremely apposite because the distinction between “cryptogenic” and “idiopathic” disease is seminal in the ongoing idiopathic pulmonary fibrosis (IPF) terminology debate.
In our recent perspective [1], we highlighted the dangers of conflating two separate aspects of pathogenesis: the initiation of disease, which may be largely related to epithelial events, and disease progression, which is likely to be influenced by the stromal response that includes fibroblasts and immune cells. Recent advances have largely related to the former and it can be argued, vide Cottin, that based on increasing knowledge of predilections and triggers, IPF is less cryptogenic than it once was: perhaps the origin of the disease is now “obscure” but increasingly less “hidden”. However, the relentless progression of fibrosis in IPF remains, in the words of Sir Winston Churchill, “a riddle, wrapped in a mystery, inside an enigma”: verily, “a disease of its own”. We agree that the term “idiopathic” aptly summarises the conundrum that is IPF progression.
Footnotes
The IPF Consensus Working Group: K.M. Antoniou, J. Behr, P.B. Bitterman, C.D. Cool, U. Costabel, T.V. Colby, V. Cottin, B. Crestani, G.P. Downey, J. Gauldie, S.D. Groshong, A. Guenther, J.C. Horowitz, Y. Inoue, J.A. Lasky, A.H. Limper, D.A. Lynch, F.J. Martinez, J.L. Myers, A.G. Nicholson, I. Noth, V. Poletti, C.J. Ryerson, J.H. Ryu, D. Valeyre, C. Vancheri and E.S. White.
Conflict of interest: A.U. Wells reports personal fees for consultancy and lecturing from Intermune/Roche, Boehringer Ingelheim and Bayer, and personal fees for consultancy from Gilead, outside the submitted work.
Conflict of interest: K.K. Brown reports multiple lung fibrosis grants from NHLBI, personal fees from AstraZeneca, Biogen, Fibrogen, Galecto, Gilead, MedImmune, Novartis, Aeolus, ProMetic, Patara, Third Pole, aTyr, Galapagos and Boehringer Ingelheim, has a submitted grant with Roche/Genentech, and conversation under CDA only with Global Blood Therapeutics and Genoa, outside the submitted work.
Conflict of interest: K.R. Flaherty reports grants and personal fees from Boehringer Ingelheim and Roche/Genentech, grants from Afferent, personal fees from Veracyte, Fibrogen, Immuneworks, Aeolus, Pharmakea, Sanofi-genzyme and Celgene, outside the submitted work.
Conflict of interest: M. Kolb reports grants and personal fees from Roche, Boehringer Ingelheim, GSK and Prometic, personal fees from Gilead and Genoa, and grants from Actelion, Respivert, Alkermes and Pharmaxis, outside the submitted work.
Conflict of interest: V.J. Thannickal has nothing to disclose.
- Received February 26, 2019.
- Accepted February 26, 2019.
- Copyright ©ERS 2019
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