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Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease

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Abstract

Purpose

Among patients with autoimmune myositis, associated interstitial lung disease (MA-ILD) is a known contributor of excess morbidity and mortality. Recent data on survival in idiopathic inflammatory myopathies originate primarily in Asia and Europe and vary widely. We sought to examine mortality in a large U.S. myositis cohort focusing in particular on the impact of associated ILD.

Methods

A cross-sectional analysis of participants from the Johns Hopkins Myositis Center with autoimmune myositis (polymyositis [PM], dermatomyositis [DM], or clinically amyopathic dermatomyositis [CADM]) was conducted. The primary outcome assessed was all-cause mortality. Cumulative mortality rates were estimated using the Kaplan–Meier test; the Cox proportional hazards model was used to compare group differences in survival.

Results

Eight hundred and thirty-one participants were included with a median follow-up time of 4.5 years. Four hundred thirty-eight (53 %) had PM, 362 (43 %) had DM, and 31 (4 %) had CADM. Ninety-four (11 %) participants had clinically evident ILD. Overall, 51 participants died (6 %). In those without ILD, the survival rates at 1, 5, and 10 years were 99, 95, and 90 %, respectively. In those with ILD, the survival rates at 1, 5, and 10 years were 97, 91, and 81 %, respectively. The risk of death was statistically significantly higher among participants with ILD compared to those without ILD (HR 2.13. 95 % CI 1.06–4.25; p = 0.03).

Conclusions

We analyzed one of the largest known cohorts of patients with autoimmune myositis and found significantly higher mortality rates among those with clinically evident ILD compared to those without clinically evident ILD. Our results suggest that ILD remains an important and significant source of mortality in patients with inflammatory myopathies and as such should be screened for and treated aggressively.

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Acknowledgments

The authors would like to thank William Kelly for his contribution to improvements in the Myositis Center Clinical Database.

Funding

This study was financially supported by The Huayi and Siuling Zhang Discovery Fund.

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Authors and Affiliations

  1. Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, 1830 E. Monument Street Suite 500, Baltimore, MD, 21224, USA

    Cheilonda Johnson & Sonye Danoff

  2. Muscle Disease Unit, Laboratory of Muscle Stem Cells and Gene Expression, National Institutes of Health, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, USA

    Iago Pinal-Fernandez & Andrew L. Mammen

  3. Division of Pulmonary and Critical Care Medicine, University of Vermont Medical Center, Burlington, VT, 05401, USA

    Radhika Parikh

  4. Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Julie Paik, Jemima Albayda & Lisa Christopher-Stine

  5. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Andrew L. Mammen

Authors
  1. Cheilonda Johnson
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  2. Iago Pinal-Fernandez
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  3. Radhika Parikh
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  4. Julie Paik
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  5. Jemima Albayda
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  6. Andrew L. Mammen
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  7. Lisa Christopher-Stine
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  8. Sonye Danoff
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Corresponding author

Correspondence to Sonye Danoff.

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The authors have no known conflicts of interest to disclose.

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Johnson, C., Pinal-Fernandez, I., Parikh, R. et al. Assessment of Mortality in Autoimmune Myositis With and Without Associated Interstitial Lung Disease. Lung 194, 733–737 (2016). https://doi.org/10.1007/s00408-016-9896-x

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  • DOI: https://doi.org/10.1007/s00408-016-9896-x

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