Fibroblast heterogeneity in pulmonary fibrosis: a new target for therapeutics development?

Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease with unknown aetiology [1]. The prevalence of this devastating disease continues to increase, affecting approximately 500 per 100 000 adults over the age of 65 years in the USA, and leading to rising rates of hospital admissions and deaths [2–5]. Two therapies for IPF approved by the US Food and Drug Administration, nintedanib and pirfenidone, can attenuate the decline in lung function. However, neither of these drugs can stop or reverse disease progression [6, 7]. Thus, an improved understanding of the pathogenesis of IPF and its underlying cellular and molecular mechanisms will facilitate the development of more effective therapies against this disorder. Do distinct fibroblast subpopulations contribute differentially to fibrosis progression and are these subsets therapeutic targets?