The ILD-GAP risk prediction model performs poorly in myositis-associated interstitial lung disease

Rebeccah M. Brusca, Iago Pinal-Fernandez, Kevin Psoter, Julie J. Paik, Jemima Albayda, Christopher Mecoli, Eleni Tiniakou, Andrew L. Mammen, Lisa Christopher-Stine, Sonye Danoff, Cheilonda Johnson

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish (US)
Pages (from-to)63-65
Number of pages3
JournalRespiratory Medicine
StatePublished - Apr 2019


  • Calibration
  • Cross-sectional studies
  • Interstitial
  • Lung diseases
  • Myositis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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