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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.

Change history

References

  1. Dall’Era, M. et al. The incidence and prevalence of systemic lupus erythematosus in San Francisco County, California: the California Lupus Surveillance Project. Arthritis Rheumatol. 69, 1996–2005 (2017).

    Article  PubMed  Google Scholar 

  2. Izmirly, P. M. et al. The Incidence and prevalence of systemic lupus erythematosus in New York County (Manhattan), New York: the Manhattan Lupus Surveillance Program. Arthritis Rheumatol. 69, 2006–2017 (2017).

    Article  PubMed  Google Scholar 

  3. Pons-Estel, G. J., Ugarte-Gil, M. F. & Alarcon, G. S. Epidemiology of systemic lupus erythematosus. Expert Rev. Clin. Immunol. 13, 799–814 (2017).

    Article  CAS  PubMed  Google Scholar 

  4. Rees, F., Doherty, M., Grainge, M. J., Lanyon, P. & Zhang, W. The worldwide incidence and prevalence of systemic lupus erythematosus: a systematic review of epidemiological studies. Rheumatology 56, 1945–1961 (2017).

    Article  PubMed  Google Scholar 

  5. Carter, E. E., Barr, S. G. & Clarke, A. E. The global burden of SLE: prevalence, health disparities and socioeconomic impact. Nat. Rev. Rheumatol. 12, 605–620 (2016).

    Article  PubMed  Google Scholar 

  6. Yen, E. Y. & Singh, R. R. Lupus — an unrecognized leading cause of death in young females: a population-based study using nationwide death certificates, 2000–2015. Arthritis Rheumatol. 70, 1251–1255 (2018).

    Article  PubMed  PubMed Central  Google Scholar 

  7. Lee, Y. H., Choi, S. J., Ji, J. D. & Song, G. G. Overall and cause-specific mortality in systemic lupus erythematosus: an updated meta-analysis. Lupus 25, 727–734 (2016).

    Article  CAS  PubMed  Google Scholar 

  8. Feldman, C. H. et al. Development of a set of lupus-specific ambulatory care sensitive, potentially preventable adverse conditions: a Delphi consensus study. Arthritis Care Res. 73, 146–157 (2019).

    Article  Google Scholar 

  9. Li, S., Gong, T., Peng, Y., Nieman, K. M. & Gilbertson, D. T. Prevalence and incidence of systemic lupus erythematosus and associated outcomes in the 2009–2016 US Medicare population. Lupus 29, 15–26 (2020).

    Article  CAS  PubMed  Google Scholar 

  10. Ungprasert, P. et al. Incidence of systemic lupus erythematosus in a population-based cohort using revised 1997 American College of Rheumatology and the 2012 Systemic Lupus International Collaborating Clinics classification criteria. Lupus 26, 240–247 (2017).

    Article  CAS  PubMed  Google Scholar 

  11. Fatoye, F., Gebrye, T. & Svenson, L. W. Real-world incidence and prevalence of systemic lupus erythematosus in Alberta, Canada. Rheumatol. Int. 38, 1721–1726 (2018).

    Article  PubMed  PubMed Central  Google Scholar 

  12. World Health Organization. International classification of diseases for mortality and morbidity statistics (11th Revision). https://icd.who.int/browse11/l-m/en (2020).

  13. Somers, E. C. et al. Population-based incidence and prevalence of systemic lupus erythematosus: the Michigan Lupus Epidemiology and Surveillance program. Arthritis Rheumatol. 66, 369–378 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  14. Lim, S. S. et al. The incidence and prevalence of systemic lupus erythematosus, 2002–2004: the Georgia Lupus Registry. Arthritis Rheumatol. 66, 357–368 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  15. Ferucci, E. D. et al. Prevalence and incidence of systemic lupus erythematosus in a population-based registry of American Indian and Alaska Native people, 2007–2009. Arthritis Rheumatol. 66, 2494–2502 (2014).

    Article  PubMed  PubMed Central  Google Scholar 

  16. Housey, M. et al. Incidence and prevalence of systemic lupus erythematosus among Arab and Chaldean Americans in southeastern Michigan: the Michigan Lupus Epidemiology and Surveillance Program. Am. J. Public Health 105, e74–e79 (2015).

    Article  PubMed  PubMed Central  Google Scholar 

  17. Yen, E. Y. et al. 46-year trends in systemic lupus erythematosus mortality in the United States, 1968 to 2013: a nationwide population-based study. Ann. Intern. Med. 167, 777–785 (2017).

    Article  PubMed  PubMed Central  Google Scholar 

  18. Lim, S. S. et al. Racial disparities in mortality associated with systemic lupus erythematosus — Fulton and DeKalb Counties, Georgia, 2002–2016. MMWR 68, 419–422 (2019).

    PubMed  PubMed Central  Google Scholar 

  19. Nee, R. et al. Survival disparity of African American versus non-African American patients with ESRD due to SLE. Am. J. Kidney Dis. 66, 630–637 (2015).

    Article  PubMed  Google Scholar 

  20. Gomez-Puerta, J. A. et al. Racial/ethnic variation in all-cause mortality among United States Medicaid recipients with systemic lupus erythematosus: a Hispanic and Asian paradox. Arthritis Rheumatol. 67, 752–760 (2015).

    Article  PubMed  PubMed Central  Google Scholar 

  21. Anastasiou, C. et al. Mortality among hospitalized individuals with systemic lupus erythematosus in the United States between 2006 and 2016. Arthritis Care Res. https://doi.org/10.1002/acr.24356 (2020).

    Article  Google Scholar 

  22. Dhital, R. et al. All-cause hospitalizations and mortality in systemic lupus erythematosus in the US: results from a national inpatient database. Rheumatol. Int. 40, 393–397 (2020).

    Article  PubMed  Google Scholar 

  23. Falasinnu, T., Chaichian, Y. & Simard, J. F. Impact of sex on systemic lupus erythematosus-related causes of premature mortality in the United States. J. Womens Health 26, 1214–1221 (2017).

    Article  Google Scholar 

  24. Brinks, R. et al. Age-specific and sex-specific incidence of systemic lupus erythematosus: an estimate from cross-sectional claims data of 2.3 million people in the German statutory health insurance 2002. Lupus Sci. Med. 3, e000181 (2016).

    Article  PubMed  PubMed Central  Google Scholar 

  25. Hermansen, M. L., Lindhardsen, J., Torp-Pedersen, C., Faurschou, M. & Jacobsen, S. Incidence of systemic lupus erythematosus and lupus nephritis in Denmark: a Nationwide Cohort Study. J. Rheumatol. 43, 1335–1339 (2016).

    Article  PubMed  Google Scholar 

  26. Kedves, M. et al. Large-scale mortality gap between SLE and control population is associated with increased infection-related mortality in lupus. Rheumatology 59, 3443–3451 (2020).

    Article  PubMed  PubMed Central  Google Scholar 

  27. Rees, F. et al. The incidence and prevalence of systemic lupus erythematosus in the UK, 1999–2012. Ann. Rheum. Dis. 75, 136–141 (2016).

    Article  PubMed  Google Scholar 

  28. Arnaud, L. et al. Prevalence and incidence of systemic lupus erythematosus in France: a 2010 nation-wide population-based study. Autoimmun. Rev. 13, 1082–1089 (2014).

    Article  PubMed  Google Scholar 

  29. Simard, J. F., Sjowall, C., Ronnblom, L., Jonsen, A. & Svenungsson, E. Systemic lupus erythematosus prevalence in Sweden in 2010: what do national registers say? Arthritis Care Res. 66, 1710–1717 (2014).

    Article  Google Scholar 

  30. Gergianaki, I. et al. Epidemiology and burden of systemic lupus erythematosus in a Southern European population: data from the community-based lupus registry of Crete, Greece. Ann. Rheum. Dis. 76, 1992–2000 (2017).

    Article  PubMed  Google Scholar 

  31. Ingvarsson, R. F., Bengtsson, A. A. & Jonsen, A. Variations in the epidemiology of systemic lupus erythematosus in southern Sweden. Lupus 25, 772–780 (2016).

    Article  CAS  PubMed  Google Scholar 

  32. Tsioni, V. et al. The prevalence and incidence of systemic lupus erythematosus in children and adults: a population-based study in a mountain community in northern Italy. Clin. Exp. Rheumatol. 33, 681–687 (2015).

    PubMed  Google Scholar 

  33. Magro, R. & Borg, A. A. Characterisation of patients with systemic lupus erythematosus in Malta: a population based cohort cross-sectional study. Biomed. Res. Int. 2018, 2385386 (2018).

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  34. Otsa, K. et al. Prevalence and incidence of systemic lupus erythematosus in the adult population of Estonia. Lupus 26, 1115–1120 (2017).

    Article  CAS  PubMed  Google Scholar 

  35. Cortes Verdu, R. et al. Prevalence of systemic lupus erythematosus in Spain: higher than previously reported in other countries? Rheumatology 59, 2556–2562 (2020).

    Article  PubMed  PubMed Central  Google Scholar 

  36. Ambrose, N. et al. Differences in disease phenotype and severity in SLE across age groups. Lupus 25, 1542–1550 (2016).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Ingvarsson, R. F., Landgren, A. J., Bengtsson, A. A. & Jonsen, A. Good survival rates in systemic lupus erythematosus in southern Sweden, while the mortality rate remains increased compared with the population. Lupus 28, 1488–1494 (2019).

    Article  CAS  PubMed  Google Scholar 

  38. Jorge, A. M., Lu, N., Zhang, Y., Rai, S. K. & Choi, H. K. Unchanging premature mortality trends in systemic lupus erythematosus: a general population-based study (1999–2014). Rheumatology 57, 337–344 (2018).

    Article  PubMed  Google Scholar 

  39. Bultink, I. E. M., de Vries, F., van Vollenhoven, R. F. & Lalmohamed, A. Mortality, causes of death and influence of medication use in patients with systemic lupus erythematosus vs matched controls. Rheumatology 60, 207–216 (2020).

    Article  PubMed Central  CAS  Google Scholar 

  40. Rees, F. et al. Mortality in systemic lupus erythematosus in the United Kingdom 1999–2012. Rheumatology 55, 854–860 (2016).

    Article  PubMed  Google Scholar 

  41. Yee, C. S. et al. Birmingham SLE cohort: outcomes of a large inception cohort followed for up to 21 years. Rheumatology 54, 836–843 (2015).

    Article  CAS  PubMed  Google Scholar 

  42. Taraborelli, M. et al. Organ damage accrual and distribution in systemic lupus erythematosus patients followed-up for more than 10 years. Lupus 26, 1197–1204 (2017).

    Article  CAS  PubMed  Google Scholar 

  43. Hermansen, M. L., Lindhardsen, J., Torp-Pedersen, C., Faurschou, M. & Jacobsen, S. The risk of cardiovascular morbidity and cardiovascular mortality in systemic lupus erythematosus and lupus nephritis: a Danish nationwide population-based cohort study. Rheumatology 56, 709–715 (2017).

    PubMed  Google Scholar 

  44. Reppe Moe, S. E., Molberg, O., Strom, E. H. & Lerang, K. Assessing the relative impact of lupus nephritis on mortality in a population-based systemic lupus erythematosus cohort. Lupus 28, 818–825 (2019).

    Article  CAS  PubMed  Google Scholar 

  45. Padjen, I. et al. Disease characteristics and causes of early and late death in a group of Croatian patients with systemic lupus erythematosus deceased over a 10-year period. Croat. Med. J. 59, 3–12 (2018).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Gonzalez Lucero, L. et al. Prevalence and incidence of systemic lupus erythematosus in Tucuman, Argentina. Lupus 29, 1815–1820 (2020).

    Article  PubMed  Google Scholar 

  47. Haase, I. et al. Incidence of systemic lupus erythematosus in Colombia: data from the National Health Registry 2012-2016. Lupus 28, 1610–1611 (2019).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Scolnik, M. et al. Incidence and prevalence of lupus in Buenos Aires, Argentina: a 11-year health management organisation-based study. Lupus Sci. Med. 1, e000021 (2014).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Fernandez-Avila, D. G., Bernal-Macias, S., Rincon-Riano, D. N., Gutierrez Davila, J. M. & Rosselli, D. Prevalence of systemic lupus erythematosus in Colombia: data from the national health registry 2012–2016. Lupus 28, 1273–1278 (2019).

    Article  CAS  PubMed  Google Scholar 

  50. Granados, Y. et al. Prevalence of musculoskeletal disorders and rheumatic diseases in an urban community in Monagas State, Venezuela: a COPCORD study. Clin. Rheumatol. 34, 871–877 (2015).

    Article  PubMed  Google Scholar 

  51. Guevara-Pacheco, S. et al. Prevalence of musculoskeletal disorders and rheumatic diseases in Cuenca, Ecuador: a WHO-ILAR COPCORD study. Rheumatol. Int. 36, 1195–1204 (2016).

    Article  PubMed  Google Scholar 

  52. Quintana, R. et al. Prevalence of musculoskeletal disorders and rheumatic diseases in the indigenous Qom population of Rosario, Argentina. Clin. Rheumatol. 35 (Suppl. 1), 5–14 (2016).

    Article  PubMed  PubMed Central  Google Scholar 

  53. Ribeiro Costi, L., Iwamoto, H. M., Neves, D. C. O. & Caldas, C. A. M. Mortality from systemic erythematosus lupus in Brazil: evaluation of causes according to the government health database. Rev. Bras. Reumatol. Engl. Ed. 57, 574–582 (2017).

    Article  Google Scholar 

  54. Souza, D. C., Santo, A. H. & Sato, E. I. Trends in systemic lupus erythematosus mortality rates in the state of Sao Paulo, Brazil from 1985 to 2004. Clin. Exp. Rheumatol. 28, 519–524 (2010).

    CAS  PubMed  Google Scholar 

  55. Lau, C. S. & Mak, A. The socioeconomic burden of SLE. Nat. Rev. Rheumatol. 5, 400–404 (2009).

    Article  PubMed  Google Scholar 

  56. Chen, H. A. et al. Predictors of longterm mortality in patients with and without systemic lupus erythematosus on maintenance dialysis: a comparative study. J. Rheumatol. 38, 2390–2394 (2011).

    Article  PubMed  Google Scholar 

  57. Chiu, Y. M. & Lai, C. H. Nationwide population-based epidemiologic study of systemic lupus erythematosus in Taiwan. Lupus 19, 1250–1255 (2010).

    Article  PubMed  Google Scholar 

  58. Shim, J. S., Sung, Y. K., Joo, Y. B., Lee, H. S. & Bae, S. C. Prevalence and incidence of systemic lupus erythematosus in South Korea. Rheumatol. Int. 34, 909–917 (2014).

    Article  PubMed  Google Scholar 

  59. Al Dhanhani, A. M., Agarwal, M., Othman, Y. S. & Bakoush, O. Incidence and prevalence of systemic lupus erythematosus among the native Arab population in UAE. Lupus 26, 664–669 (2017).

    Article  CAS  PubMed  Google Scholar 

  60. Zou, Y. F. et al. Prevalence of systemic lupus erythematosus and risk factors in rural areas of Anhui Province. Rheumatol. Int. 34, 347–356 (2014).

    Article  PubMed  Google Scholar 

  61. Yeh, K. W., Yu, C. H., Chan, P. C., Horng, J. T. & Huang, J. L. Burden of systemic lupus erythematosus in Taiwan: a population-based survey. Rheumatol. Int. 33, 1805–1811 (2013).

    Article  PubMed  Google Scholar 

  62. Al Durahim, H., Al Ghamdi, G., Al Seraya, A., Alkhiari, R. & Al Sayyari, A. Predictors of mortality and end stage renal disease in Saudi patients with lupus nephritis. Lupus 20, 1329–1335 (2011).

    Article  CAS  PubMed  Google Scholar 

  63. Chen, Y. M. et al. Onset age affects mortality and renal outcome of female systemic lupus erythematosus patients: a nationwide population-based study in Taiwan. Rheumatology 53, 180–185 (2014).

    Article  PubMed  Google Scholar 

  64. Dhir, V., Aggarwal, A., Lawrence, A., Agarwal, V. & Misra, R. Long-term outcome of lupus nephritis in Asian Indians. Arthritis Care Res. 64, 713–720 (2012).

    Article  Google Scholar 

  65. Fatemi, A. et al. Outcome of adult onset systemic lupus erythematosus in Iran. Lupus 23, 1211–1216 (2014).

    Article  CAS  PubMed  Google Scholar 

  66. Foocharoen, C., Nanagara, R., Suwannaroj, S. & Mahakkanukrauh, A. Survival rate among Thai systemic lupus erythematosus patients in the era of aggressive treatment. Int. J. Rheum. Dis. 14, 353–360 (2011).

    Article  PubMed  Google Scholar 

  67. Feng, X. et al. Prognostic indicators of hospitalized patients with systemic lupus erythematosus: a large retrospective multicenter study in China. J. Rheumatol. 38, 1289–1295 (2011).

    Article  PubMed  Google Scholar 

  68. Kono, M. et al. Long-term outcome in Japanese patients with lupus nephritis. Lupus 23, 1124–1132 (2014).

    Article  CAS  PubMed  Google Scholar 

  69. Joo, Y. B. & Bae, S. C. Assessment of clinical manifestations, disease activity and organ damage in 996 Korean patients with systemic lupus erythematosus: comparison with other Asian populations. Int. J. Rheum. Dis. 18, 117–128 (2015).

    Article  PubMed  Google Scholar 

  70. Hwang, J. et al. Clinical characteristics of male and female Korean patients with systemic lupus erythematosus: a comparative study. Korean J. Intern. Med. 30, 242–249 (2015).

    Article  PubMed  PubMed Central  Google Scholar 

  71. Lin, H. et al. Survival analysis of late-onset systemic lupus erythematosus: a cohort study in China. Clin. Rheumatol. 31, 1683–1689 (2012).

    Article  PubMed  Google Scholar 

  72. Mok, C. C., Kwok, R. C. & Yip, P. S. Effect of renal disease on the standardized mortality ratio and life expectancy of patients with systemic lupus erythematosus. Arthritis Rheum. 65, 2154–2160 (2013).

    Article  CAS  PubMed  Google Scholar 

  73. Wu, X. Y. et al. Causes of death in hospitalized patients with systemic lupus erythematosus: a 10-year multicenter nationwide Chinese cohort. Clin. Rheumatol. 38, 107–115 (2019).

    Article  PubMed  Google Scholar 

  74. Jakes, R. W. et al. Systematic review of the epidemiology of systemic lupus erythematosus in the Asia-Pacific region: prevalence, incidence, clinical features, and mortality. Arthritis Care Res. 64, 159–168 (2012).

    Article  Google Scholar 

  75. Lewis, M. J. & Jawad, A. S. The effect of ethnicity and genetic ancestry on the epidemiology, clinical features and outcome of systemic lupus erythematosus. Rheumatology 56, i67–i77 (2017).

    Article  CAS  PubMed  Google Scholar 

  76. Mak, A., Cheung, M. W., Chiew, H. J., Liu, Y. & Ho, R. C. Global trend of survival and damage of systemic lupus erythematosus: meta-analysis and meta-regression of observational studies from the 1950s to 2000s. Semin. Arthritis Rheum. 41, 830–839 (2012).

    Article  PubMed  Google Scholar 

  77. Anstey, N. M., Bastian, I., Dunckley, H. & Currie, B. J. Systemic lupus erythematosus in Australian aborigines: high prevalence, morbidity and mortality. Aust. N. Z. J. Med. 23, 646–651 (1993).

    Article  CAS  PubMed  Google Scholar 

  78. Segasothy, M. & Phillips, P. A. Systemic lupus erythematosus in Aborigines and Caucasians in central Australia: a comparative study. Lupus 10, 439–444 (2001).

    Article  CAS  PubMed  Google Scholar 

  79. Grennan, D. M. & Bossingham, D. Systemic lupus erythematosus (SLE): different prevalences in different populations of Australian aboriginals. Aust. N. Z. J. Med. 25, 182–183 (1995).

    Article  CAS  PubMed  Google Scholar 

  80. Bossingham, D. Systemic lupus erythematosus in the far north of Queensland. Lupus 12, 327–331 (2003).

    Article  CAS  PubMed  Google Scholar 

  81. Hart, H. H., Grigor, R. R. & Caughey, D. E. Ethnic difference in the prevalence of systemic lupus erythematosus. Ann. Rheum. Dis. 42, 529–532 (1983).

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  82. Hochberg, M. C. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 40, 1725 (1997).

    Article  CAS  PubMed  Google Scholar 

  83. Petri, M. et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 64, 2677–2686 (2012).

    Article  PubMed  PubMed Central  Google Scholar 

  84. Aringer, M. et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann. Rheum. Dis. 78, 1151–1159 (2019).

    Article  PubMed  Google Scholar 

  85. Bae, S. C., Fraser, P. & Liang, M. H. The epidemiology of systemic lupus erythematosus in populations of African ancestry: a critical review of the “prevalence gradient hypothesis”. Arthritis Rheum. 41, 2091–2099 (1998).

    Article  CAS  PubMed  Google Scholar 

  86. Gilkeson, G. et al. The United States to Africa lupus prevalence gradient revisited. Lupus 20, 1095–1103 (2011).

    Article  PubMed  PubMed Central  Google Scholar 

  87. Tiffin, N., Hodkinson, B. & Okpechi, I. Lupus in Africa: can we dispel the myths and face the challenges? Lupus 23, 102–111 (2014).

    Article  CAS  PubMed  Google Scholar 

  88. Vento, S. & Cainelli, F. Commentary: systemic lupus erythematosus in native sub-Saharan Africans: a systematic review and meta-analysis. Front. Med. 7, 202 (2020).

    Article  Google Scholar 

  89. Essouma, M. et al. Systemic lupus erythematosus in Native sub-Saharan Africans: a systematic review and meta-analysis. J. Autoimmun. 106, 102348 (2020).

    Article  CAS  PubMed  Google Scholar 

  90. Bongomin, F., Sekimpi, M. & Kaddumukasa, M. Concise Report Clinical and immunological characteristics of 56 patients with systemic lupus erythematosus in Uganda. Rheumatol. Adv. Practice 4, rkaa011 (2020).

    Article  Google Scholar 

  91. Bija, M. D. et al. Clinical presentation, treatment and outcome of patients with systemic lupus erythematosus seen at a rheumatology clinic in Douala, Cameroon. Health Sci. Dis. 15, 1–5 (2014).

    Google Scholar 

  92. Edet, M., Otike-Odibi, B. & Altraide, D. Profile of autoimmune connective tissue disorders in the University of Port Harcourt teaching hospital, Port Harcourt, Nigera. Nigerian Health J. 15, 39–47 (2015).

    Google Scholar 

  93. Ekwom, P. E. Systemic lupus erythematosus (SLE) at the Kenyatta National Hospital. Clin. Rheumatol. 32, 1215–1217 (2013).

    Article  PubMed  Google Scholar 

  94. Gbané-Koné, M. et al. Autoantibodies in systemic lupus erythematosus, on Black African subject, in Abidjan. Open J. Rheumatol. Autoimmune Dis. 05, 28–35 (2015).

    Article  Google Scholar 

  95. Genga, E. K. et al. Clinical characteristics of patients with systemic lupus erythematosus in Nairobi, Kenya. Afr. J. Rheumatol. 3, 62–66 (2015).

    Google Scholar 

  96. Kane, B. S. et al. Systemic diseases in Dakar (Senegal): spectrum, epidemiological aspect and diagnostic time-limit. Open J. Intern. Med. 08, 196–206 (2018).

    Article  Google Scholar 

  97. Ayodele, O. E., Okpechi, I. G. & Swanepoel, C. R. Long-term renal outcome and complications in South Africans with proliferative lupus nephritis. Int. Urol. Nephrol. 45, 1289–1300 (2013).

    Article  PubMed  Google Scholar 

  98. Budhoo, A., Mody, G. M., Dubula, T., Patel, N. & Mody, P. G. Comparison of ethnicity, gender, age of onset and outcome in South Africans with systemic lupus erythematosus. Lupus 26, 438–446 (2017).

    Article  CAS  PubMed  Google Scholar 

  99. Dubula, T. & Mody, G. M. Spectrum of infections and outcome among hospitalized South Africans with systemic lupus erythematosus. Clin. Rheumatol. 34, 479–488 (2015).

    Article  PubMed  Google Scholar 

  100. Dzifa, D. et al. Predictors and outcome of systemic lupus erythematosus (SLE) admission rates in a large teaching hospital in sub-Saharan Africa. Lupus 27, 336–342 (2018).

    Article  CAS  PubMed  Google Scholar 

  101. Brower, V. Counterfeit and substandard malaria drugs in Africa. Lancet. Infect. Dis. 17, 1026–1027 (2017).

    Article  PubMed  Google Scholar 

  102. Banerjee, Y. Counterfeit and substandard drugs in sub-Saharan Africa may pose a major hurdle to H3Africa’s initiative to study genetics of kidney disease progression. Kidney Int. 91, 252–253 (2017).

    Article  PubMed  Google Scholar 

  103. Consortium, H. A. et al. Research capacity. Enabling the genomic revolution in Africa. Science 344, 1346–1348 (2014).

    Article  Google Scholar 

  104. Collaborators, G. B. D. R. F. Global burden of 87 risk factors in 204 countries and territories, 1990–2019: a systematic analysis for the Global Burden of Disease Study 2019. Lancet 396, 1223–1249 (2020).

    Article  Google Scholar 

  105. Scherlinger, M. et al. Worldwide trends in all-cause mortality of auto-immune systemic diseases between 2001 and 2014. Autoimmun. Rev. 19, 102531 (2020).

    Article  CAS  PubMed  Google Scholar 

  106. Tektonidou, M. G., Lewandowski, L. B., Hu, J., Dasgupta, A. & Ward, M. M. Survival in adults and children with systemic lupus erythematosus: a systematic review and Bayesian meta-analysis of studies from 1950 to 2016. Ann. Rheum. Dis. 76, 2009–2016 (2017).

    Article  PubMed  Google Scholar 

  107. Choi, M. Y. et al. Antinuclear antibody–negative systemic lupus erythematosus in an international inception cohort. Arthritis Care Res. 71, 893–902 (2019).

    Article  CAS  Google Scholar 

  108. Leuchten, N. et al. Performance of antinuclear antibodies for classifying systemic lupus erythematosus: a systematic literature review and meta-regression of diagnostic data. Arthritis Care Res. 70, 428–438 (2018).

    Article  CAS  Google Scholar 

  109. Askanase, A. D. et al. Development and content validity of the Lupus Foundation of America rapid evaluation of activity in lupus (LFA-REAL): a patient-reported outcome measure for lupus disease activity. Health Qual. Life Outcomes 17, 99 (2019).

    Article  PubMed  PubMed Central  Google Scholar 

  110. Fries, J. F. & Holman, H. R. Systemic lupus erythematosus: a clinical analysis. Major Probl. Intern. Med. 6, v-199 (1975).

    PubMed  Google Scholar 

  111. World Health Organization. Global Health Observatory data repository http://apps.who.int/gho/data/view.main.LT62020? (2019).

  112. Bernatsky, S. et al. Mortality in systemic lupus erythematosus. Arthritis Rheum. 54, 2550–2557 (2006).

    Article  CAS  PubMed  Google Scholar 

  113. Tan, E. M. et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 25, 1271–1277 (1982).

    Article  CAS  PubMed  Google Scholar 

Download references

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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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