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

Julie Morisset1, Eric Vittinghoff2, Brett M Elicker3

  • 1Department of Medicine, University of California, San Francisco, San Francisco, CA.

Chest
|June 21, 2017
PubMed
Summary

A new SADL model predicts mortality in scleroderma-associated interstitial lung disease (Scl-ILD) using smoking history, age, and lung function. This tool aids risk stratification for better patient management.

Keywords:
interstitial lung diseaseprognosisrisk predictionsystemic sclerosis

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Area of Science:

  • Pulmonary Medicine
  • Rheumatology
  • Clinical Epidemiology

Background:

  • Scleroderma-associated interstitial lung disease (Scl-ILD) significantly contributes to patient morbidity and mortality.
  • Predicting risk and prognosis in Scl-ILD is difficult due to disease heterogeneity.

Purpose of the Study:

  • To develop and validate a clinical model for predicting mortality risk in patients with Scl-ILD.

Main Methods:

  • A mortality risk prediction model was developed using a derivation cohort (n=135) and validated in an independent cohort (n=90).
  • Cox models were tested with 3-4 variables from 19 potential clinical predictors.
  • Model discrimination was assessed using the C-index.

Main Results:

  • The final SADL model incorporates ever smoking history, age, and diffusing capacity of the lung for carbon monoxide (predicted %)
  • The SADL model demonstrated strong performance in both derivation (C-index=0.88) and validation (C-index=0.84) cohorts.
  • A scoring system derived from the combined cohort (C-index=0.82) classified patients into low, moderate, and high 3-year mortality risk categories.

Conclusions:

  • The SADL model provides a simple and accessible tool for predicting all-cause mortality in Scl-ILD.
  • This model can aid clinicians in risk stratification and management of Scl-ILD patients.