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The fibrosing IIPs are characterised by progressive scarring of the alveolar interstitium, which leads to significant morbidity and mortality. Idiopathic pulmonary fibrosis (IPF) is the most common and most severe form of IIP with a median survival of 3 years, limited treatment options and an increasing prevalence. The strongest and most replicated risk factor for IPF is the promoter variant located in a highly conserved region of the genome ~3 kilobases upstream of the airway mucin MUC5B transcription start site.1 The MUC5B variant rs35705950 has a regulatory role and is associated with increased RNA expression in whole lung tissue in IPF and controls.2 In mice, transgenic overexpression …
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests IVY is a consultant for Eleven P15.
Patient consent for publication Not required.
Provenance and peer review Commissioned; externally peer reviewed.
Data availability statement No data are available. Not applicable to commentary article.
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