Prognostic significance of 1-year pulmonary function changes in inflammatory myopathy-related interstitial lung disease
Issued Date
2026-06-01
Resource Type
ISSN
00490172
eISSN
1532866X
Scopus ID
2-s2.0-105034655254
Pubmed ID
41903313
Journal Title
Seminars in Arthritis and Rheumatism
Volume
78
Rights Holder(s)
SCOPUS
Bibliographic Citation
Seminars in Arthritis and Rheumatism Vol.78 (2026)
Suggested Citation
Keret S., Laverde S.M., Silva R.L., Choudhuri I., Gkiaouraki E., Chandra T., Pongtarakulpanit N., Bhowmick N., Kothari V., Reddy K.S., Alhassan E., Aggarwal A., Almackenzie M., Sullivan D.I., Faghihi-Kashani S., Yamaguchi K., Kass D., Gibson K., Ascherman D.P., Moghadam-Kia S., Oddis C.V., Aggarwal R. Prognostic significance of 1-year pulmonary function changes in inflammatory myopathy-related interstitial lung disease. Seminars in Arthritis and Rheumatism Vol.78 (2026). doi:10.1016/j.semarthrit.2026.152957 Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/116054
Title
Prognostic significance of 1-year pulmonary function changes in inflammatory myopathy-related interstitial lung disease
Author's Affiliation
Massachusetts General Hospital
University of Pittsburgh School of Medicine
Duke University School of Medicine
University of Pittsburgh Medical Center
Graduate School of Medicine
Faculty of Medicine Ramathibodi Hospital, Mahidol University
Bnai Zion Medical Center
Indraprastha Apollo Hospitals
Ministry of Interior
University of Pittsburgh School of Medicine
Duke University School of Medicine
University of Pittsburgh Medical Center
Graduate School of Medicine
Faculty of Medicine Ramathibodi Hospital, Mahidol University
Bnai Zion Medical Center
Indraprastha Apollo Hospitals
Ministry of Interior
Corresponding Author(s)
Other Contributor(s)
Abstract
BackgroundThe prognostic value of pulmonary function test (PFT) trends in idiopathic inflammatory myopathy-related interstitial lung disease (IIM-ILD) remains unclear. We evaluated whether one-year changes in forced vital capacity (FVC) and diffusing capacity (DLCO) predict 10-year mortality and lung transplantation.MethodsIn a retrospective, single-center cohort of adults with IIM-ILD classified by autoantibody status and 2017 EULAR/ACR criteria, ILD was defined by high-resolution chest CT (HRCT). Inclusion required baseline and follow-up PFTs 6–18 months apart. Cox regression and Kaplan-Meier analyses assessed associations between PFT changes and survival. Multivariable models adjusted for age, sex, smoking, baseline FVC, and PFT timing.ResultsThe most common IIM subset among 149 patients (mean age 50.5 ± 12.9 years, 63 % female) was anti-synthetase syndrome (73 %). Over mean 6.3-year follow-up, 41 (27.5 %) died and 6 (4.0 %) underwent transplantation. In multivariate analyses, absolute and relative FVC declines of ≥5% over one year were significantly associated with increased 10-year mortality (HR=2.78, CI 1.27–6.09, p = 0.01 and HR=2.37, CI 1.11–5.05, p = 0.025). Larger FVC declines (≥10%/≥15 %) showed stronger mortality associations, whereas stable or improved FVC predicted better outcomes (HR=0.38, CI 0.18–0.82, p = 0.01). DLCO decline was not associated with survival. Kaplan-Meier analysis demonstrated worse survival with FVC decline≥5 % (p = 0.028). Survival did not differ by autoantibody subtype or HRCT pattern.ConclusionEven modest FVC decline (≥5 %) over one year predicts mortality and transplant in IIM-ILD, while stabilization or improvement in FVC associates with improved survival and should be considered therapeutic goals. Routine FVC monitoring may support risk stratification, guide transplant referral, and serve as a trial endpoint.