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  • Review Article
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Global epidemiology of systemic lupus erythematosus

Nature Reviews Rheumatology volume 17, pages 515–532 (2021)Cite this article

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A Publisher Correction to this article was published on 01 September 2021

This article has been updated

Abstract

Systemic lupus erythematosus (SLE) is an autoimmune disease with protean manifestations that predominantly affects young women. Certain ethnic groups are more vulnerable than others to developing SLE and experience increased morbidity and mortality. Reports of the global incidence and prevalence of SLE vary widely, owing to inherent variation in population demographics, environmental exposures and socioeconomic factors. Differences in study design and case definitions also contribute to inconsistent reporting. Very little is known about the incidence of SLE in Africa and Australasia. Identifying and remediating such gaps in epidemiology is critical to understanding the global burden of SLE and improving patient outcomes. Mortality from SLE is still two to three times higher than that of the general population. Internationally, the frequent causes of death for patients with SLE include infection and cardiovascular disease. Even without new therapies, mortality can potentially be mitigated with enhanced quality of care. This Review focuses primarily on the past 5 years of global epidemiological studies and discusses the regional incidence and prevalence of SLE and top causes of mortality.

Key points

  • The estimated incidence, prevalence and mortality of systemic lupus erythematosus (SLE) vary considerably between geographic regions.

  • Factors that contribute to the variation across different regions include differences in ethnicity, environmental exposures and socioeconomic status but non-uniform SLE definitions and study design also contribute.

  • Population-based studies in the developing world are urgently needed to understand the global burden of disease.

  • Mortality in patients with SLE is still unacceptably high, being two to three times higher than that of the general population.

  • Infectious diseases and cardiovascular disease are consistently top causes of death in patients with SLE.

  • Even without the development of new therapies, SLE outcomes may be improved by focusing on remediable SLE-specific adverse conditions.

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Fig. 1: Global incidence estimates for SLE.
Fig. 2: Global prevalence estimates for SLE.

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  1. Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada

    Megan R. W. Barber & Ann E. Clarke

  2. Department of Medicine, Division of Rheumatology, Emory University School of Medicine, Atlanta, GA, USA

    Cristina Drenkard

  3. Department of Anesthesiology, Perioperative and Pain Medicine, Stanford School of Medicine, Stanford, CA, USA

    Titilola Falasinnu

  4. School of Clinical Sciences at Monash Health, Monash University, Melbourne, Victoria, Australia

    Alberta Hoi

  5. Division of Rheumatology, University Medicine Cluster, National University Health System, Singapore, Singapore

    Anselm Mak & Nien Yee Kow

  6. Department of Medicine, Yong Loo Lin School of Medicine, National University Hospital, Singapore, Singapore

    Anselm Mak & Nien Yee Kow

  7. Department of Medicine Solna, Unit of Rheumatology, Karolinska Institute, Karolinska University Hospital, Stockholm, Sweden

    Elisabet Svenungsson

  8. Galter Health Sciences Library and Learning Center, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Jonna Peterson

  9. Department of Medicine, Rheumatology Division, Feinberg School of Medicine, Northwestern University, Chicago, IL, USA

    Rosalind Ramsey-Goldman

Authors
  1. Megan R. W. Barber
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  2. Cristina Drenkard
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  3. Titilola Falasinnu
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  4. Alberta Hoi
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  5. Anselm Mak
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  6. Nien Yee Kow
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  7. Elisabet Svenungsson
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  8. Jonna Peterson
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  9. Ann E. Clarke
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  10. Rosalind Ramsey-Goldman
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Contributions

R.R.-G., M.R.W.B., C.D., T.F., A.H., A.M., N.Y.K., E.S. and J.P. researched data for the article; R.R.-G., M.R.W.B. and A.E.C. made substantial contributions to discussions of the content; M.R.W.B., C.D., T.F., A.H., A.M., N.Y.K. and E.S. wrote the article; and R.R.-G., M.R.W.B. and A.E.C. reviewed and edited the manuscript before submission.

Corresponding author

Correspondence to Rosalind Ramsey-Goldman.

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Competing interests

A.E.C. has received consulting fees from AstraZeneca, Exagen Diagnostics and GlaxoSmithKline. A.M. has received consulting fees from Janssen and research grants from GlaxoSmithKline and Janssen. C.D. is supported by a GlaxoSmithKline award (ID 0000048412). E.S. has received honoraria from Janssen and Lilly. R.R.-G. has received research grants from Exagen Diagnostics and consulting fees from Thermo Fisher Scientific and Aurinia Pharmaceuticals. A.H. has received honoraria from AstraZeneca, Janssen and AbbVie, and research grant from Merck Serono. M.R.W.B has received consulting fees from AbbVie, Janssen, GlaxoSmithKline and Sanofi Genzyme. All other authors declare no competing interests.

Additional information

Peer review information

Nature Reviews Rheumatology thanks G. Pons-Estel, who co-reviewed with R. Quintana; and G. Bertsias for their contribution to the peer review of this work.

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Review criteria

A comprehensive search was developed by J.P. in conjunction with the other authors. The search terms used for this project were “systemic lupus erythematosus”, “mortality”, “incidence”, “prevalence” and “socioeconomic factors”. Terms were also compiled for the geographic regions of interest (by continent): North America, South America, Asia, Australia, Africa and Europe. The searches were performed in MEDLINE via PubMed (1966–present). Search results were limited to those items published in the English language and using the MEDLINE human filter. The initial strategy was to include data from the past 5 years, but to represent areas where resources might be limited older studies were included when they were informative. The authors also used additional references of particular interest that were not included in the formal search.

Supplementary information

Glossary

Prevalence

The total number of cases of disease in a given timeframe.

Incidence

The number of new cases of disease during a specified timeframe.

Capture-recapture methodology

A method that determines the extent of overlap across multiple case-finding sources to adjust for case under-ascertainment.

Point prevalence

The prevalence of a disease at a specified point in time.

Jaccoud arthropathy

A form of chronic, non-erosive, reducible arthropathy that can affect patients with systemic lupus erythematosus.

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Barber, M.R.W., Drenkard, C., Falasinnu, T. et al. Global epidemiology of systemic lupus erythematosus. Nat Rev Rheumatol 17, 515–532 (2021). https://doi.org/10.1038/s41584-021-00668-1

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