SLE Epidemiology: Epidemiologic Subtypes and Risk Factors for Development

  • Julia F. Simard
  • Karen H. Costenbader


While the etiology of systemic lupus erythematosus (SLE) is still enigmatic, epidemiologic research is continuously informing our understanding, with new insights into environmental risk factors and gene–environment interactions that play a role in disease susceptibility. SLE is a heterogeneous multisystem autoimmune disorder characterized by organ system manifestations which vary over time and autoantibodies that target mainly intracellular constituents. Antinuclear antibodies (ANA) are observed in over 95% of patients, while other characteristic autoantibodies [anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), anti-Ro, and anti-La antibodies] are less common. These autoantibodies have been detected years prior to first symptoms of SLE and are responsible for certain disease manifestations such as photosensitive rashes, cytopenias, thromboses, and glomerulonephritis.


Systemic Lupus Erythematosus Systemic Lupus Erythematosus Patient Systemic Lupus Erythematosus Disease Activity Undifferentiated Connective Tissue Disease Silicone Breast Implant 
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  1. 1.
    Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725.PubMedCrossRefGoogle Scholar
  2. 2.
    Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, Schaller JG, Talal N, Winchester RJ. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25(11):1271–7.PubMedCrossRefGoogle Scholar
  3. 3.
    Costenbader KH, Karlson EW, Mandl LA. Defining lupus cases for clinical studies: the Boston weighted criteria for the classification of systemic lupus erythematosus. J Rheumatol. 2002;29(12):2545–50.PubMedGoogle Scholar
  4. 4.
    Petri M. Review of classification criteria for systemic lupus erythematosus. Rheum Dis Clin North Am. 2005;31(2):245–54. vi.PubMedCrossRefGoogle Scholar
  5. 5.
    To CH, Petri M. Is antibody clustering predictive of clinical subsets and damage in systemic lupus erythematosus? Arthritis Rheum. 2005;52(12):4003–10.PubMedCrossRefGoogle Scholar
  6. 6.
    Brunner HI, Gladman DD, Ibanez D, Urowitz MD, Silverman ED. Difference in disease features between childhood-onset and adult-onset systemic lupus erythematosus. Arthritis Rheum. 2008;58(2):556–62.PubMedCrossRefGoogle Scholar
  7. 7.
    Tucker LB, Uribe AG, Fernandez M, Vila LM, McGwin G, Apte M, Fessler BJ, Bastian HM, Reveille JD, Alarcon GS. Adolescent onset of lupus results in more aggressive disease and worse outcomes: results of a nested matched case-control study within LUMINA, a multiethnic US cohort (LUMINA LVII). Lupus. 2008;17(4):314–22.PubMedCrossRefGoogle Scholar
  8. 8.
    Sanchez-Guerrero J, Villegas A, Mendoza-Fuentes A, Romero-Diaz J, Moreno-Coutino G, Cravioto MC. Disease activity during the premenopausal and postmenopausal periods in women with systemic lupus erythematosus. Am J Med. 2001;111(6):464–8.PubMedCrossRefGoogle Scholar
  9. 9.
    McCarty DJ, Manzi S, Medsger Jr TA, Ramsey-Goldman R, LaPorte RE, Kwoh CK. Incidence of systemic lupus erythematosus. Race and gender differences. Arthritis Rheum. 1995;38(9):1260–70.PubMedCrossRefGoogle Scholar
  10. 10.
    Alarcon GS, Friedman AW, Straaton KV, Moulds JM, Lisse J, Bastian HM, McGwin G, Jr., Bartolucci AA, Roseman JM, Reveille JD. Systemic lupus erythematosus in three ethnic groups: III. A comparison of characteristics early in the natural history of the LUMINA cohort. LUpus in MInority populations: NAture vs. Nurture. Lupus. 1999;8(3):197–209.PubMedCrossRefGoogle Scholar
  11. 11.
    Vasudevan A, Krishnamurthy AN. Changing worldwide epidemiology of systemic lupus erythematosus. Rheum Dis Clin North Am. 2010;36(1):1–13. vii.PubMedCrossRefGoogle Scholar
  12. 12.
    Danchenko N, Satia JA, Anthony MS. Epidemiology of systemic lupus erythematosus: a comparison of worldwide disease burden. Lupus. 2006;15(5):308–18.PubMedCrossRefGoogle Scholar
  13. 13.
    Bae SC, Fraser P, Liang MH. The epidemiology of systemic lupus erythematosus in populations of African ancestry: a critical review of the “prevalence gradient hypothesis”. Arthritis Rheum. 1998;41(12):2091–9.PubMedCrossRefGoogle Scholar
  14. 14.
    Costenbader KH, Feskanich D, Stampfer MJ, Karlson EW. Reproductive and menopausal factors and risk of systemic lupus erythematosus in women. Arthritis Rheum. 2007;56(4):1251–62.PubMedCrossRefGoogle Scholar
  15. 15.
    Bernier MO, Mikaeloff Y, Hudson M, Suissa S. Combined oral contraceptive use and the risk of systemic lupus erythematosus. Arthritis Rheum. 2009;61(4):476–81.PubMedCrossRefGoogle Scholar
  16. 16.
    Costenbader KH, Kim DJ, Peerzada J, Lockman S, Nobles-Knight D, Petri M, Karlson EW. Cigarette smoking and the risk of systemic lupus erythematosus: a meta-analysis. Arthritis Rheum. 2004;50:849–57.PubMedCrossRefGoogle Scholar
  17. 17.
    Cooper GS, Dooley MA, Treadwell EL, St Clair EW, Gilkeson GS. Smoking and use of hair treatments in relation to risk of developing systemic lupus erythematosus. J Rheumatol. 2001;28(12):2653–6.PubMedGoogle Scholar
  18. 18.
    Cooper GS, Wither J, Bernatsky S, Claudio JO, Clarke A, Rioux JD, Fortin PR. Occupational and environmental exposures and risk of systemic lupus erythematosus: silica, sunlight, solvents. Rheumatology. 2010;49(11):2172–80.PubMedCrossRefGoogle Scholar
  19. 19.
    Parks CG, Walitt BT, Pettinger M, Chen JC, De Roos AJ, Hunt J, Sarto G, Howard BV. Insecticide use and risk of rheumatoid arthritis and systemic lupus erythematosus in the women’s health initiative observational study. Arth Care Res. 2011;63(2):184–94.CrossRefGoogle Scholar
  20. 20.
    Parks CG, Cooper GS, Nylander-French LA, Sanderson WT, Dement JM, Cohen PL, Dooley MA, Treadwell EL, St Clair EW, Gilkeson GS, Hoppin JA, Savitz DA. Occupational exposure to crystalline silica and risk of systemic lupus erythematosus: a population-based, case-control study in the southeastern United States. Arthritis Rheum. 2002;46(7):1840–50.PubMedCrossRefGoogle Scholar
  21. 21.
    Finckh A, Cooper GS, Chibnik LB, Costenbader KH, Fraser PA, Watts J, Pankey H, Karlson EW. Occupational silica and solvent exposures and risk of systemic lupus erythematosus in urban women. Arthritis Rheum. 2006;54(11):3648–54.PubMedCrossRefGoogle Scholar
  22. 22.
    Sanchez-Guerrero J, Karlson EW, Colditz GA, Hunter DJ, Speizer FE, Liang MH. Hair dye use and the risk of developing systemic lupus erythematosus. Arthritis Rheum. 1996;39(4):657–62.PubMedCrossRefGoogle Scholar
  23. 23.
    Sutcliffe N, Clarke AE, Gordon C, Farewell V, Isenberg DA. The association of socio-economic status, race, psychosocial factors and outcome in patients with systemic lupus erythematosus. Rheumatology (Oxford). 1999;38(11):1130–7.CrossRefGoogle Scholar
  24. 24.
    Duran S, Apte M, Alarcon GS. Poverty, not ethnicity, accounts for the differential mortality rates among lupus patients of various ethnic groups. J Natl Med Assoc. 2007;99(10):1196–8.PubMedGoogle Scholar
  25. 25.
    Costenbader KH, Feskanich D, Holmes M, Karlson EW, Benito-Garcia E. Vitamin D intake and risks of systemic lupus erythematosus and rheumatoid arthritis in women. Ann Rheum Dis. 2008;67(4):530–5.PubMedCrossRefGoogle Scholar
  26. 26.
    Costenbader KH, Kang JH, Karlson EW. Antioxidant intake and risks of rheumatoid arthritis and systemic lupus erythematosus in women. Am J Epidemiol. 2010;172(2):205–16.PubMedCrossRefGoogle Scholar
  27. 27.
    Bengtsson AA, Rylander L, Hagmar L, Nived O, Sturfelt G. Risk factors for developing systemic lupus erythematosus: a case-control study in southern Sweden. Rheumatology (Oxford). 2002;41(5):563–71.CrossRefGoogle Scholar
  28. 28.
    James JA, Neas BR, Moser KL, Hall T, Bruner GR, Sestak AL, Harley JB. Systemic lupus erythematosus in adults is associated with previous Epstein-Barr virus exposure. Arthritis Rheum. 2001;44(5):1122–6.PubMedCrossRefGoogle Scholar
  29. 29.
    Peralta-Ramirez MI, Jimenez-Alonso J, Godoy-Garcia JF, Perez-Garcia M. The effects of daily stress and stressful life events on the clinical symptomatology of patients with lupus erythematosus. Psychosom Med. 2004;66(5):788–94.PubMedCrossRefGoogle Scholar
  30. 30.
    Buyon JP, Petri MA, Kim MY, Kalunian KC, Grossman J, Hahn BH, Merrill JT, Sammaritano L, Lockshin M, Alarcon GS, Manzi S, Belmont HM, Askanase AD, Sigler L, Dooley MA, Von Feldt J, McCune WJ, Friedman A, Wachs J, Cronin M, Hearth-Holmes M, Tan M, Licciardi F. The effect of combined estrogen and progesterone hormone replacement therapy on disease activity in systemic lupus erythematosus: a randomized trial. Ann Intern Med. 2005;142(12 Pt 1):953–62.PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2012

Authors and Affiliations

  1. 1.Clinical Epidemiology UnitT2, Karolinska University HospitalStockholmSweden
  2. 2.Lupus CenterBrigham and Women’s HospitalBostonUSA
  3. 3.Harvard Medical SchoolBostonUSA

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