Year: 2024
Type: article
Abstract: Rationale Some with interstitial lung abnormalities (ILA) are suspected to have interstitial lung disease (ILD), a subgroup with adverse outcomes. Rates of development and progression of suspected ILD and their effect on mortality are unknown. Objectives To determine rates of development, progression, and mortality in those with suspected ILD and assess effects of individual ILD and progression criteria. Methods Participants from COPDGene (Genetic Epidemiology of Chronic Obstructive Pulmonary Disease) with ILA characterization and FVC at enrollment and 5-year follow-up were included. ILD was defined as ILA and fibrosis and/or FVC < 80% predicted. Prevalent ILD was assessed at enrollment and incident ILD and progression were assessed at 5-year follow-up. Computed tomography (CT) progression was assessed visually and FVC decline as relative change. Multivariable Cox regression tested associations between mortality and prevalent ILD, incident ILD, and progression groups. Measurements and Main Results Of 9,588 participants at enrollment, 268 (2.8%; 51% of ILA) had prevalent ILD. Those with prevalent ILD had 51% mortality after median 10.6 years, which was higher than those with ILA without prevalent ILD (henceforth ILA) (33%; hazard ratio [HR], 2.0; P < 0.001). The subgroup of prevalent ILD with only fibrosis criteria (FVC ≥ 80%) had worse mortality (58%) than ILA (HR, 2.2; P < 0.001). A total of 98 participants with prevalent ILD completed 5-year follow-up: 33% had stable CT and relative FVC decline <10%, 6% had FVC decline ≥10% only, 39% had CT progression only, and 22% had both CT progression and FVC decline ≥10%. Mortality rates were 31%, 50%, 45%, and 45%, respectively; those with only CT progression had worse mortality than those with ILA (HR, 2.6; P = 0.005). At 5-year follow-up, incident ILD occurred in 148/4,842 participants without prevalent ILD (5.5/1,000 person-years) and had worse mortality than ILA (HR, 2.4; P < 0.001). Conclusions Rates of mortality and progression are high among those with suspected ILD in COPDGene; fibrosis and radiologic progression are important predictors of mortality.
Authors: Jonathan A. Rose, Ann-Marcia C. Tukpah, Claire Cutting, Noriaki Wada, Mizuki Nishino, Matthew Moll, Sean Kalra, Bina Choi, David A. Lynch, Benjamin A. Raby, Ivan O. Rosas, Raúl San José Estépar, George R. Washko, Edwin K. Silverman, Michael H. Cho, Hiroto Hatabu, Rachel K. Putman, Gary M. Hunninghake
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: green
Awards: G1200984374, G1311591790, G1321641812, G1478064418, G1761310282, G2644038179, Genetic and Functional Dissection of a Cluster of COPD GWAS Signals on Chromosome 4q, G377726100, G3998889914, G437020959, Interstitial Lung Abnormalities: Defining the Phenotype, Causes, and Consequences., G5258357286, G5333041877, G554584989, G573304077, G5960470374, G6011549775, G6024979, G6179922603, Identifying Protein-Protein Network Interactions between COPD Susceptibility Genes, G737045378, G7381400632, Clinical Implications, Genomics, and Transcriptions of Mucus Plugging in Smokers, G8354759825, G9000000961, G9000002950, G9000007807, G9000010287, G9000045715, G9000071188, G9000091268, G9000108939, G9000130393, G9000166014, G9000175120, G9000183370, G9000219965, G9000220348, G9000228787, G9000351102, Integrated Training in Respiratory Research, COPD Susceptibility, Heterogeneity, and Progression: Proteomics and Genetics
OpenAlex ID: W4401511364