Extract
A recent paper by Wolters et al. [1] has raised considerable interest and stirred some debate regarding whether or not the word “idiopathic” should still be part of the name of the disease that we currently call idiopathic pulmonary fibrosis (IPF) [2]. The authors stated that the term idiopathic no longer seemed to describe this progressive lung disease accurately, especially considering the increasing understanding of the causes and pathogenesis of IPF. In a perspective published in the European Respiratory Journal, Wells et al. [3] responded that IPF remains a truly idiopathic fibrotic disease, with no overall explanation for the development of disease, with the exception of genetic predilection and some familial cases.
Abstract
“Cryptogenic” and “idiopathic” have different roots and slightly different meanings, which could reconcile the seemingly opposing views on terminology for idiopathic pulmonary fibrosis http://ow.ly/mvMb30nc2te
Footnotes
Conflict of interest: V. Cottin reports receiving the following, outside the submitted work: personal fees and non-financial support from Actelion, Boehringer Ingelheim and Roche for consultancy, lectures and travel to meetings; grants to his institution from Boehringer Ingelheim and Roche; personal fees from Bayer/MSD and GSK for consultancy; personal fees from Gilead for acting as a member of an adjudication committee; personal fees from Novartis for consultancy and lectures; a grant from Sanofi for acting as a trial investigator; personal fees from Promedior for acting as the Chair of the DSMB; personal fees from Celgene for the DSMB; and personal fees from Galapagos for consultancy.
- Received December 5, 2018.
- Accepted December 6, 2018.
- Copyright ©ERS 2019