Mortality Risk Prediction in Scleroderma-Related Interstitial Lung Disease: The SADL Model

Julie Morisset, Eric Vittinghoff, Brett M. Elicker, Xiaowen Hu, Stephanie Le, Jay H. Ryu, Kirk D. Jones, Anna Haemel, Jeffrey A. Golden, Francesco Boin, Brett Ley, Paul J. Wolters, Talmadge E. King, Harold R. Collard, Joyce S. Lee

Research output: Contribution to journalArticlepeer-review

31 Scopus citations


Background Interstitial lung disease (ILD) is an important cause of morbidity and mortality in patients with scleroderma (Scl). Risk prediction and prognostication in patients with Scl-ILD are challenging because of heterogeneity in the disease course. Methods We aimed to develop a clinical mortality risk prediction model for Scl-ILD. Patients with Scl-ILD were identified from two ongoing longitudinal cohorts: 135 patients at the University of California, San Francisco (derivation cohort) and 90 patients at the Mayo Clinic (validation cohort). Using these two separate cohorts, a mortality risk prediction model was developed and validated by testing every potential candidate Cox model, each including three or four variables of a possible 19 clinical predictors, for time to death. Model discrimination was assessed using the C-index. Results Three variables were included in the final risk prediction model (SADL): ever smoking history, age, and diffusing capacity of the lung for carbon monoxide (% predicted). This continuous model had similar performance in the derivation (C-index, 0.88) and validation (C-index, 0.84) cohorts. We created a point scoring system using the combined cohort (C-index, 0.82) and used it to identify a classification with low, moderate, and high mortality risk at 3 years. Conclusions The SADL model uses simple, readily accessible clinical variables to predict all-cause mortality in Scl-ILD.

Original languageEnglish (US)
Pages (from-to)999-1007
Number of pages9
Issue number5
StatePublished - Nov 2017


  • interstitial lung disease
  • prognosis
  • risk prediction
  • systemic sclerosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine


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