Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry

Nathan Hambly, M Malik Farooqi, Anna Dvorkin-Gheva, Kathryn Donohoe, Kristopher Garlick, Ciaran Scallan, Sy Giin Chong, Sarah Macisaac, Deborah Assayag, Kerri A Johannson, Charlene D Fell, Veronica Marcoux, Helene Manganas, Julie Morisset, Alessia Comes, Jolene H Fisher, Shane Shapera, Andrea S Gershon, Teresa To, Alyson W WongMohsen Sadatsafavi, Pierce G Wilcox, Andrew J Halayko, Nasreen Khalil, Gerard Cox, Luca Richeldi, Christopher J Ryerson, Martin Kolb

Risultato della ricerca: Contributo in rivistaArticolo in rivista


Rationale Progressive fibrosing interstitial lung disease (PF-ILD) is characterized by progressive physiologic, symptomatic, and/or radiographic worsening. The real-world prevalence and characteristics of PF-ILD remain uncertain. Methods Patients were enrolled from the Canadian Registry for Pulmonary Fibrosis between 2015-2020. PF-ILD was defined as a relative forced vital capacity (FVC) decline ≥10%, death, lung transplantation, or any 2 of: relative FVC decline ≥5 and <10%, worsening respiratory symptoms, or worsening fibrosis on computed tomography of the chest, all within 24 months of diagnosis. Time-to-event analysis compared progression between key diagnostic subgroups. Characteristics associated with progression were determined by multivariable regression. Results Of 2,746 patients with fibrotic ILD (mean age 65±12 years, 51% female), 1,376 (50%) met PFILD criteria in the first 24 months of follow-up. PF-ILD occurred in 427 (59%) patients with idiopathic pulmonary fibrosis (IPF), 125 (58%) with fibrotic hypersensitivity pneumonitis (HP), 281 (51%) with unclassifiable ILD (U-ILD), and 402 (45%) with connective tissue diseaseassociated ILD (CTD-ILD). Compared to IPF, time to progression was similar in patients with HP (hazard ratio [HR] 0.96, 95% confidence interval, CI 0.79-1.17), but was delayed in patients with U-ILD (HR 0.82, 95% CI 0.71-0.96) and CTD-ILD (HR 0.65, 95% CI 0.56-0.74). Background treatment varied across diagnostic subtypes with 66% of IPF patients receiving antifibrotic therapy, while immunomodulatory therapy was utilized in 49%, 61%, and 37% of patients with CHP, CTD-ILD, and U-ILD respectively. Increasing age, male sex, gastroesophageal reflux disease, and lower baseline pulmonary function were independently associated with progression. Interpretation Progression is common in patients with fibrotic ILD, and is similarly prevalent in HP and IPF. Routinely collected variables help identify patients at risk for progression and may guide therapeutic strategies
Lingua originaleEnglish
pagine (da-a)2102571-2102580
Numero di pagine10
RivistaEuropean Respiratory Journal
Stato di pubblicazionePubblicato - 2022


  • progressive fibrosing interstitial lung disease


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