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Assessing Mortality Models in Systemic Sclerosis-Related Interstitial Lung Disease

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Abstract

Purpose

The gender, age, and lung physiology (GAP) model, interstitial lung diseases—GAP (ILD-GAP) model, and the smoking history, age, and diffusion capacity of the lung (SADL) model were compared using a systemic sclerosis-ILD (SSc-ILD) cohort to evaluate which best determined prognosis.

Methods

The models were applied to a cohort of 179 patients with SSc seen at a tertiary care center within 1 year of ILD diagnosis. Demographics, clinical characteristics, and mortality were recorded. The performance of the models was assessed using standardized mortality ratios (SMR) of observed versus predicted outcomes for calibration and concordance (c)-statistics for discrimination.

Results

SSc-ILD patients with usual interstitial pneumonia (31, 17%) had a higher mortality than those with non-specific interstitial pneumonia (147, 83%) (hazard ratio 2.27; 95%CI 1.03–4.97). All 3 models had comparable discrimination (c = 0.72, 0.72, and 0.71, respectively). Regarding calibration, the ILD-GAP model underestimated mortality (SMR 1.50; 95%CI 1.05–2.14). Calibration was acceptable for SADL (SMR 1.00; 95%CI 0.70–1.44) and GAP (SMR 0.90; 95%CI 0.63–1.29). The SADL model underestimated mortality in Stage I ILD.

Conclusions

The ILD-GAP model underestimated mortality, and the SADL model underestimated mortality in certain subgroups. However, the GAP model performed well in this cohort, providing the best prognostic information for SSc-ILD.

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Acknowledgements

This publication was made possible by CTSA Grant Number UL1 TR000135 from the National Center for Advancing Translational Sciences (NCATS), a component of the National Institutes of Health (NIH). Its contents are solely the responsibility of the authors and do not necessarily represent the official view of NIH.

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Authors and Affiliations

  1. Division of Rheumatology, Mayo Clinic College of Medicine and Science, Rochester, MN, USA

    Robert L. Mango, Eric L. Matteson, Cynthia S. Crowson & Ashima Makol

  2. Department of Health Sciences Research, Mayo Clinic College of Medicine and Science, Rochester, MN, USA

    Eric L. Matteson & Cynthia S. Crowson

  3. Division of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine and Science, Rochester, MN, USA

    Jay H. Ryu

  4. Division of Rheumatology, Department of Internal Medicine, Mayo Clinic, 200 1st St SW, Rochester, MN, 55905, USA

    Ashima Makol

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  1. Robert L. Mango
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Correspondence to Ashima Makol.

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Mango, R.L., Matteson, E.L., Crowson, C.S. et al. Assessing Mortality Models in Systemic Sclerosis-Related Interstitial Lung Disease. Lung 196, 409–416 (2018). https://doi.org/10.1007/s00408-018-0126-6

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  • DOI: https://doi.org/10.1007/s00408-018-0126-6

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