TY - JOUR
T1 - Prevalence and characteristics of progressive fibrosing interstitial lung disease in a prospective registry
AU - Hambly, Nathan
AU - Farooqi, M. Malik
AU - Dvorkin-Gheva, Anna
AU - Donohoe, Kathryn
AU - Garlick, Kristopher
AU - Scallan, Ciaran
AU - Chong, Sy Giin
AU - Macisaac, Sarah
AU - Assayag, Deborah
AU - Johannson, Kerri A.
AU - Fell, Charlene D.
AU - Marcoux, Veronica
AU - Manganas, Helene
AU - Morisset, Julie
AU - Comes, Alessia
AU - Fisher, Jolene H.
AU - Shapera, Shane
AU - Gershon, Andrea S.
AU - To, Teresa
AU - Wong, Alyson W.
AU - Sadatsafavi, Mohsen
AU - Wilcox, Pierce G.
AU - Halayko, Andrew J.
AU - Khalil, Nasreen
AU - Cox, Gerard
AU - Richeldi, Luca
AU - Ryerson, Christopher J.
AU - Kolb, Martin
PY - 2022
Y1 - 2022
N2 - 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
AB - 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
KW - progressive fibrosing interstitial lung disease
KW - progressive fibrosing interstitial lung disease
UR - http://hdl.handle.net/10807/203448
U2 - 10.1183/13993003.02571-2021
DO - 10.1183/13993003.02571-2021
M3 - Article
SN - 0903-1936
VL - 2022
SP - 2102571
EP - 2102580
JO - European Respiratory Journal
JF - European Respiratory Journal
ER -