Rebeccah M Brusca1, Iago Pinal-Fernandez2, Kevin Psoter1, Julie J Paik1, Jemima Albayda1, Christopher Mecoli1, Eleni Tiniakou1, Andrew L Mammen2, Lisa Christopher-Stine1, Sonye Danoff1, Cheilonda Johnson3. 1. Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 500, Baltimore, MD, 21224, USA. 2. Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 500, Baltimore, MD, 21224, USA; National Institute of Arthritis and Musculoskeletal and Skin Disease, National Institute of Health, Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Regulation, 9000 Rockville Pike Building 50, Bethesda, MD, 20892, USA. 3. Johns Hopkins University School of Medicine, 1830 East Monument Street, Suite 500, Baltimore, MD, 21224, USA. Electronic address: Cheilonda.Johnson@uphs.upenn.edu.
Abstract
PURPOSE: Myositis-associated interstitial lung disease (MA-ILD) is associated with increased mortality, but no prognostic model exists in this population. The ILD-GAP index was developed to predict mortality risk across all subtypes of chronic ILD. The purpose of this study was to validate the ILD-GAP risk prediction model in patients with MA-ILD. PROCEDURES: We completed a retrospective cross-sectional study of patients enrolled in the Johns Hopkins Myositis Center database between 2006 and 2017. Cumulative mortality rates were estimated using the Kaplan-Meier test. Model calibration was determined by using standardized mortality ratios of observed versus expected deaths. MAIN FINDINGS: 179 participants with MA-ILD were included. The mean baseline percent predicted forced vital capacity was 65.2 ± 20.6%, forced expiratory volume in the first second 65.4 ± 20.4%, and carbon monoxide diffusing capacity 61.6 ± 20.0%. Thirty-two participants died (17.9%). The ILD-GAP model had poor discriminative performance and calibration. CONCLUSIONS: The ILD-GAP risk prediction model is a poor predictor of mortality among individuals with MA-ILD. The identification of a better predictive model for MA-ILD is needed to help guide care in this patient population.
PURPOSE:Myositis-associated interstitial lung disease (MA-ILD) is associated with increased mortality, but no prognostic model exists in this population. The ILD-GAP index was developed to predict mortality risk across all subtypes of chronic ILD. The purpose of this study was to validate the ILD-GAP risk prediction model in patients with MA-ILD. PROCEDURES: We completed a retrospective cross-sectional study of patients enrolled in the Johns Hopkins Myositis Center database between 2006 and 2017. Cumulative mortality rates were estimated using the Kaplan-Meier test. Model calibration was determined by using standardized mortality ratios of observed versus expected deaths. MAIN FINDINGS: 179 participants with MA-ILD were included. The mean baseline percent predicted forced vital capacity was 65.2 ± 20.6%, forced expiratory volume in the first second 65.4 ± 20.4%, and carbon monoxide diffusing capacity 61.6 ± 20.0%. Thirty-two participants died (17.9%). The ILD-GAP model had poor discriminative performance and calibration. CONCLUSIONS: The ILD-GAP risk prediction model is a poor predictor of mortality among individuals with MA-ILD. The identification of a better predictive model for MA-ILD is needed to help guide care in this patient population.
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