Abstract
Rationale Progressive fibrosing interstitial lung disease (PF-ILD) is characterised 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 2746 patients with fibrotic ILD (mean age 65±12 years, 51% female), 1376 (50%) met PF-ILD 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 disease-associated 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 utilised 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.
Footnotes
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Conflict of interest: NH reports grants from Roche, Boehringer Ingelheim; personal fees from Roche, Boehringer Ingelheim, Janssen.
Conflict of interest: MF reports no conflicts of interest relevant to this manuscript.
Conflict of interest: ADG reports no conflicts of interest relevant to this manuscript.
Conflict of interest: KD reports no conflicts of interest relevant to this manuscript.
Conflict of interest: KG was an employee for the medical department of Boehringer Ingelheim at the time of the preparation of this manuscript.
Conflict of interest: CS reports personal fees from Boehringer Ingelheim.
Conflict of interest: SGC reports no conflicts of interest relevant to this manuscript.
Conflict of interest: SM reports no conflicts of interest relevant to this manuscript.
Conflict of interest: DA reports personal fees from Roche and Boehringer Ingelheim.
Conflict of interest: KAJ reports grants from Boehringer Ingelheim, Pulmonary Fibrosis Society of Calgary, University of Calgary School of Medicine; personal fees from Boehringer Ingelheim, Roche, Three Lakes Foundation, Pliant Therapeutics, Theravance, Blade Therapeutics.
Conflict of interest: CDF reports educational grants and research grants from Boehringer-Ingelheim, Roche and the Canadian Pulmonary Fibrosis Foundation; personal fees from Roche and Boehringer Ingelheim; Chair of the Boards for the Canadian Pulmonary Fibrosis Foundation.
Conflict of interest: VM reports grants from Boehringer Ingelheim, Astra Zeneca, and Roche; personal fees from Boehringer Ingelheim and Roche.
Conflict of interest: HM reports grants from Boehringer Ingelheim and Gilead.
Conflict of interest: JM reports personal fees from Boehringer Ingelheim and Roche.
Conflict of interest: AC reports no conflicts of interest relevant to this manuscript.
Conflict of interest: JHF reports no conflicts of interest relevant to this manuscript.
Conflict of interest: SS reports educational grants and research grants from Boehringer-Ingelheim, Roche, and the Canadian Pulmonary Fibrosis Foundation; personal fees from Astra-Zeneca, Boehringer-Ingelheim Canada and Hoffman La-Roche Canada; served on the medical advisory board for the Canadian Pulmonary Fibrosis Foundation.
Conflict of interest: ASG reports a research grant from the Canadian Pulmonary Fibrosis Foundation.
Conflict of interest: TT reports no conflicts of interest relevant to this manuscript.
Conflict of interest: AWW reports personal fees from AstraZeneca and Boehringer Ingelheim.MS reports grants from Boehringer Ingelheim; personal fees from Boehringer Ingelheim.
Conflict of interest: PGW reports personal fees from Boehringer Ingelheim.
Conflict of interest: AJH reports no conflicts of interest relevant to this manuscript.
Conflict of interest: NK reports no conflicts of interest relevant to this manuscript.
Conflict of interest: GC reports personal fees from personal fees from Boehringer Ingelheim and Roche.
Conflict of interest: LR reports grants from personal fees from Boehringer Ingelheim; personal fees from Biogen, Sanofi-Aventis, Celgene, RespiVant, CSL Behring, Nitto, Pliant Therapeutics, Cipla, Zambon, Promedior, Boehringer Ingelheim, Roche, Fibrogen.
Conflict of interest: CJR reports grants from Boehringer Ingelheim; personal fees from Boehringer Ingelheim, Roche, Pliant Therapeutics, Cipla, Veracyte.
Conflict of interest: MK reports grants from Canadian Institute for Health Research, Roche, Boehringer Ingelheim, Pieris, Prometic; personal fees from Boehringer Ingelheim, Roche, European Respiratory Journal, Belerophon, United Therapeutics, Nitto Denko, MitoImmune, Pieris, Abbvie, DevPro Biopharma, Horizon, Algernon, CSL Behring.
- Received September 27, 2021.
- Accepted February 17, 2022.
- Copyright ©The authors 2022. For reproduction rights and permissions contact permissions{at}ersnet.org
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