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Systemic Lupus Erythematosus (SLE)

This is an overview of the epidemiology of systemic lupus erthematosus (SLE). You can find basic information on the CDC Lupus website.

Systemic lupus erythematosus (SLE) is an autoimmune disease in which the immune system makes antibodies to cells in the body leading to widespread inflammation and tissue damage. The causes of SLE are unknown but are believed to be linked to genetic, environmental, and hormonal factors. SLE may have periods of illness and remission. SLE has a variety of signs and symptoms and can affect the joints, skin, brain, lungs, kidneys, and blood vessels. People with SLE may experience fatigue, pain or swelling in joints, skin rashes, and fevers. A team approach in treating SLE is often needed because of the number of organ systems that can be involved.

I. Background

  • SLE is a prototypical autoimmune disease with a wide array of signs and symptoms (rash, sun sensitivity, oral ulcers, arthritis, lung problems, heart problems, kidney problems, seizures and psychosis, blood cell abnormalities). It is characterized by the production of antibodies to components of the cell nucleus.1 
  • Primarily a disease of young women. 1  
  • Occurs from infancy to old age, with peak in the childbearing years for females.1
  • Females are affected far more than males (4 to12 females for every male).1
  • Blacks (and probably Hispanics, Asians, and Native Americans) are affected more than whites.1
  • People with a family history of SLE or other autoimmune diseases are at slightly higher risk for developing SLE. This risk increases as the number of first-degree family members with SLE increases1 but even so most cases are sporadic and not familial.
  • SLE may occur with other autoimmune conditions (e.g., thyroiditis, hemolytic anemia, idiopathic thrombocytopenia purpura).1 
  • Diagnosis can be very difficult. The gold standard is a rheumatologist’s diagnosis. The American College of Rheumatology (ACR) uses a standard classification scheme requiring 4 of 11 criteria for a research definition, although this is recognized to miss early and mild cases. Even so, there is:
    • underdiagnosis because the presenting symptoms and signs are often not specific and not recognized as SLE;1 and
    • overdiagnosis because doctors mistakenly use a positive blood test (present in 5% of the healthy population) by itself to make a diagnosis.
  • Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine and corticosteroids [prednisone]). In 2011 the FDA approved the first new drug for SLE in more than 50 years—belimumab.
  • Morbidity and mortality may be related to late diagnosis, problems in access to care, less effective treatments, and poor adherence to therapeutic regimens.1


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II. Prevalence (all cases)

  • Older national prevalence estimates vary widely, and range as high as 1,500,000.2 Another study estimated a prevalence of 161,000 with definite SLE and 322,000 with definite or probable SLE.3
  • Newer and more complete CDC-funded population-based SLE registries with a primary purpose of generating better prevalence (and incidence) estimates published results in 2014.  Annual prevalence from 2002-2004 was much higher for blacks than whites in Michigan (Washtenaw and Wayne Counties) (111.6 vs 47.5 per 100,000 persons)4 and in Georgia (DeKalb and Fulton Counties) (128.0 vs. 39.9 per 100,000 persons).5 Annual prevalence from 2007-2009 for American Indians/Alaska Natives was 178 per 100,000.6  New registries in California (San Francisco County) and New York City (Manhattan) will soon provide annual prevalence estimates for Hispanics and Asians.
  • Annual prevalence estimates were much higher among women than men in Michigan (9.3 vs 1.5 per 100,000 persons),5 in Georgia (145.8 vs 17.5 per 100,000 persons),5 and in the American Indian/Alaska Native population (271 vs. 54 per 100,000 persons).6 

III. Incidence rates (new cases/year)

  • National incidence data are difficult to obtain because the diagnosis is difficult for providers and the year of onset is hard to determine (non-specific symptoms and signs), so resource-intense studies must be done in small areas.1
  • Over time incidence rates in whites in Rochester, Minnesota (Mayo Clinic’s Rochester Epidemiology Project) tripled from 1.5 cases per 100,000 persons per year in the 1950–1979 cohort to 5.6 cases per 100,000 person per year in the 1980–1992 cohort.7
  • Newer and more complete CDC-funded population-based SLE registries with a primary purpose of generating better incidence (and prevalence) estimates published results in 2014.  Annual incidence from 2002-2004 was much higher for blacks than whites in Michigan (7.9 vs. 3.7 100,000 persons)4, and in Georgia (9.4 vs. 3.2 per 100,000 persons).5 Annual incidence from 2007-2009 for American Indians/Alaska Natives was 7.4 per 100,000)6. New registries in California (San Francisco County) and New York City (Manhattan) will soon provide annual incidence estimates for Hispanics and Asians.
  • Annual incidence estimates were much higher for women than men in Michigan (9.3 vs. 1.5 per 100,000)4, Georgia (10.6 vs. 1.9 per 100,000)5 and the American Indian/Alaska Native population (unadjusted 8.4 vs. 2.7 per 100,000).6

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IV. Mortality

  • From 1979 to 1998, the annual number of deaths in the U.S. with SLE as the underlying cause increased from 879 to 1,406. Crude death rates increased with age (35% were in 15-44 year age group), among women (5x higher than in men), and among blacks (3x higher than in whites). Death rates were highest and increased the most over time among black women aged 45-64 years.8
  • An equal number listed SLE as a contributing cause of death.8
  • Causes of death are mainly active disease, organ failure (e.g., kidneys), infection, or cardiovascular disease from accelerated atherosclerosis.8
  • Among rheumatic conditions, SLE has a relatively high mortality (14.5% of all rheumatic disease mortality in 1997).8
  • In a large international SLE cohort with average follow-up of over 8 years during a 1958–2001 observation interval, observed deaths were much higher than expected for all causes, and in particular for circulatory disease, infections, renal disease, and some cancers.  Those who were female, younger, and had SLE of short duration were at higher risk.9

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V. Hospitalizations

  • There are few estimates for hospitalizations due specifically to SLE. However, data for diffuse connective tissue disease, which includes SLE, was used to estimate 39,400 hospitalizations among adults in 2010.10

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VI. Ambulatory Care

  • There are few estimates for ambulatory care visits due specifically to SLE. However, data for diffuse connective tissue disease, which includes SLE, was used to estimate 2,430,000 ambulatory care visits among adults in 2010.10

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VII. Costs

  • No national cost estimates exist specifically for SLE. However, the average hospital charges for diffuse connective tissue disease, which includes SLE, was about $13.3 billion US dollars in 2011.10
  • Costs attributable strictly to SLE are likely substantial, but credible estimates are needed from better studies that address the very difficult methodological barriers encountered.11

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VIII. Impact on Health-related Quality of Life (HRQOL)

  • Health related quality of life may be worse in patients with SLE compared to other chronic diseases. It has been difficult to find generalizable data on SLE health related quality of life because the instruments used for measurement lack disease specific sensitivity15. Despite this fact, fatigue12,13 is the most common health related quality of life outcome affected by SLE. 12
  • Employment is a measure that many studies use to determine quality of life on a global scale, because it can be important to activities that are central to a person’s life.14 Some studies have shown that as the duration of SLE disease increases, the percentage of those with SLE working decreases.  On average only 46 percent of persons with SLE reported being employed.13

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IX. Unique characteristics

  • There had been no new FDA-approved treatments for SLE since the 1950s until the 2011 approval of belimumab.1
  • Compliance/adherence to medicine regimens are often a problem, given the use of strong immunosuppressive medications and resulting side effects in young, reproductive age women who want to partner and have children. This is a relatively unique demographic/therapeutic problem among the rheumatic diseases.1
  • Patients with SLE have an increased frequency of other autoimmune problems, such as Sjogren’s syndrome (i.e., dry eyes, dry mouth) and antiphospholipid syndrome (i.e., clotting problems, strokes, fetal loss) that require additional treatments.1
  • Patients can have symptoms and signs of SLE and other conditions (e.g., scleroderma,polymyositis) called mixed connective tissue disease.1
  • Other types of lupus that are not SLE include:
    • Cutaneous lupus  (skin lupus) is lupus that affects the skin in the form of a rash or lesions. This type of lupus can occur on any part of the body, but usually appears where the skin is exposed to sunlight. Treatment for skin lupus can range from using sunscreen and wearing sun-protective clothing to using topical ointments/creams such as tacrolimus and pimecrolimus.14
    • Drug-induced lupus  is similar to SLE; however the body is attacking healthy tissues as an overreaction to certain medications. Symptoms usually occur 3 to 6 months after starting a medication, and disappear once the medicine is stopped.15
    • Neonatal lupus occurs because of passively acquired auto-antibodies from a mother with SLE. The skin, liver, and blood problems resolve by 6 months, but the most serious sign—congenital heart block—requires a pacemaker and has a mortality rate of about 20%.16

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X. References

  1. Dall’Era M.  Chapter 21. Systemic lupus erythematosus. In: Imboden JB, Hellman DB, Stone JH. (Eds). Current Rheumatology Diagnosis and Treatment. 3rd ed. New York, NY:McGraw-Hill; 2013. Accessed March 5, 2015.
  2. Lawrence RC, Felson DT, Helmick CG, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: Part II. Arthritis Rheum. 2008;58(1):26-35. abstract 
  3. Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: Part I. Arthritis Rheum. 2008;58(1):15-25. PubMed PMID: 18163481. doi: 10.1002/art.23177. abstract
  4. Somers EC, Marder W, Cagnoli P, et al. Population-based Incidence and Prevalence of Systemic Lupus Erythematosus: The Michigan Lupus Epidemiology and Surveillance Program. Arthritis Rheumatol. 2014;66(2):369-378. doi:10.102/art.38238. PubMed PMID: 24504809; PubMed Central PMCID: PMC4198147. abstract
  5. Lim SS, Bayakly AR, Helmick CG, Gordon C, Easley KA, Drenkard C. The Incidence and Prevalence of Systemic Lupus Erythematosus, 2002–2004: The Georgia Lupus Registry. Arthritis Rheumatol. 2014;66(2):357-368. doi:10.1002/art.38239. PubMed PMID: 24504808. abstract 
  6. Ferucci ED, Johnston JM, Gaddy JR, 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. 2014;66(9):2494-2502. doi: 10.1002/art.38720. PubMed PMID: 24891315. abstract
  7. Uramoto KM, Michet CJ Jr, Thumboo J, Sunku J, O'Fallon WM, Gabriel SE. Trends in the incidence and mortality of systemic lupus erythematosus, 1950–1992. Arthritis Rheum. 1999;42(1):46-50. PubMed PMID: 9920013. abstract   
  8. Sacks JJ, Helmick CG, Langmaid G, Sniezek JE. Trends in deaths from systemic lupus erythematosus—United States, 1979–1998. Morb Mortal Wkly Rep. 2002;51(17):371-374. PubMed PMID: 12018384. abstract Republished in JAMA. 2002;287(20): 2649-2650. PubMed PMID: 12035789.
  9. Bernatsky S, Boivin J-F, Joseph L, et al. Mortality in systemic lupus erythematosus. Arthritis Rheum. 2006;54:2550-2557. doi: 10.1002/art.21955. PubMed PMID: 16868977. abstract
  10. United States Bone and Joint Initiative. Arthritis and Related Conditions. In: The Burden of Musculoskeletal Diseases in the United States. 3rd ed. Rosemont, IL: United States Bone and Joint Initiative, 2014: Chapter 4.
  11. Slawsky, K. A., Fernandes, A. W., Fusfeld, L., Manzi, S. and Goss, T. F. (2011), A structured literature review of the direct costs of adult systemic lupus erythematosus in the US. Arthritis Care Res (Hoboken), 63:1224-1232. doi: 10.1002/acr.20502. PubMed PMID: 21584945. abstract
  12. Jolly M, Pickard SA, Mikolaitis RA, Rodby RA, Sequeira W, and Block JA. LupusQoL-US Benchmarks for US Patients with Systemic Lupus Erythematosus. J Rheumatol. 2010;37(9):1828-1833. PubMed PMID: 20716659. doi:10.3899/jrheum.091443. PubMed PMID: 20716659. abstract
  13. Yazdany J, and Yelin E. Health-Related Quality of Life and Employment Among Persons with Systemic Lupus Erythematosus. Rheum Dic Clin North Am. 2010;36(1):15-32. PubMed PMID: 20202589; PubMedCentral PMCID: PMC2833285. doi:10.1016/j.rdc.2009.12.006. abstract
  14. Lupus and your body: How does Lupus affect the skin? Lupus Foundation of America. Medically reviewed July 12, 2013. Accessed February 27, 2015.
  15. MedlinePlus [Internet]. Bethesda (MD): National Library of Medicine (US); [updated 2005 Aug 12]. Drug-induced lupus erythematosus; [updated 2013 April 20] Available from:]
  16. Sammaritano LR. Pregnancy in Rheumatic Disease Patients. J Clin Rheumatol. 2013;19(5):259-266. PubMed PMID: 23884185. doi: 10.1097/RHU.0b013e31829ce35f.

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XI. Resources

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