• 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, San José EstéparRaúlR0000-0002-3677-1996Brigham and Women's Hospital, Radiology, Somerville, Massachusetts, United States., George R Washko, Edwin K Silverman, Michael H Cho, Hiroto Hatabu, Rachel K Putman, and Gary M Hunninghake.
• Brigham and Women's Hospital, Pulmonary and Critical Care Division, Boston, Massachusetts, United States.
• Am. J. Respir. Crit. Care Med. 2024 Aug 12.

RationaleSome with interstitial lung abnormalities (ILA) have suspected interstitial lung disease (ILD), a subgroup with adverse outcomes. Rates of development and progression of suspected ILD and their effect on mortality are unknown.ObjectivesTo determine rates of development and progression of suspected ILD and assess effects of individual ILD and progression criteria on mortality.MethodsParticipants from COPDGene were included. ILD was defined as ILA and fibrosis and/or FVC <80% predicted. Prevalent ILD was assessed at enrollment, incident ILD and progression at 5-year follow-up. CT progression was assessed visually and FVC decline as relative change. Multivariable Cox regression tested associations between mortality and ILD groups.ResultsOf 9,588 participants at enrollment, 267 (2.8%) had prevalent ILD. Those with prevalent ILD had 52% mortality after median 10.6 years, which was higher than ILA (33%; HR=2.0; p<0.001). The subgroup of prevalent ILD with fibrosis only had worse mortality (59%) than ILA (HR=2.2; p<0.001). 97 participants with prevalent ILD completed 5-year follow-up: 32% had stable CT and relative FVC decline <10%, 6% FVC decline ≥10% only, 39% CT progression only, and 22% both CT progression and FVC decline ≥10%. Mortality rates were 32%, 50%, 45%, and 46% respectively; those with CT progression only had worse mortality than ILA (HR=2.6; p=0.005). At 5-year follow-up, incident ILD occurred in 168/4,843 participants without prevalent ILD and had worse mortality than ILA (HR=2.5; p<0.001).ConclusionRates of mortality and progression are high among those with suspected ILD in COPDGene; fibrosis and radiologic progression are important predictors of mortality.

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