Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? — wolters 2018

Idiopathic pulmonary fibrosis (IPF) is a lung condition marked by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. It is gradual, irreversible, and typically deadly. Evidence suggests that distal airway and alveolar epithelial cells are major contributors to the disease, despite the fact that our understanding of the pathogenic pathways is still developing. In order to comprehend the clinical appearance, underlying causes, and pathogenesis of the disease, we review the history of names and classifications used to describe the condition now known as IPF in this Viewpoint. We want to start a conversation on whether a name change should be taken into consideration given the significant advances achieved in understanding the clinical, genetic, cellular, and molecular factors involved in the development of IPF. To start off this conversation, we provide fresh ideas for the renaming of this illness and brand-new schemes for categorizing all types of pulmonary fibrosis.

Wolters PJ, Blackwell TS, Eickelberg O, Loyd JE, Kaminski N, Jenkins G, Maher TM, Molina-Molina M, Noble PW, Raghu G, Richeldi L, Schwarz MI, Selman M, Wuyts WA, Schwartz DA. Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic? Lancet Respir Med. 2018 Feb;6(2):154-160. doi: 10.1016/S2213-2600(18)30007-9. PMID: 29413083; PMCID: PMC5903445.