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Systemic lupus erythematosus (SLE or lupus)

Systemic Lupus Erythematosus is an autoimmune disease in which the immune system produces antibodies to cells within 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 be characterized by periods of illness and remissions. SLE has a variety of clinical manifestations and can affect 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 lupus is often warranted due to the number of organ systems involved.

I. Background

  • This report is on systemic lupus erythematosus (for shorthand, SLE or lupus), and not on 2 other types of lupus: discoid lupus (skin only), and drug-induced lupus (temporary). (1,2)
  • Lupus is a prototypical autoimmune disease with a wide array of clinical manifestations (rash, photosensitivity, oral ulcers, arthritis, pleuritis, pericarditis, kidney problems, seizures and psychosis, blood cell abnormalities). It is characterized by the production of antibodies to components of the cell nucleus. (1,2)
  • Primarily a disease of young women. (1,2)
  • Occurs from infancy to old age, with peak occurrence between ages 15 and 40. (1,2)
  • Females are affected far more than males (6-10:1). (1,2)
  • Blacks (and possibly Hispanics, Asians, and Native Americans) are affected more than whites. (1,2)
  • Although there is a strong familial aggregation, the disease is relatively uncommon and most cases are sporadic. (1,2)
  • May occur with other autoimmune conditions (e.g., thyroiditis, hemolytic anemia, idiopathic thrombocytopenia purpura). (1,2)
  • 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 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. (1,2)
    • Overdiagnosis because doctors mistakenly use a positive blood test (present in 5% of the healthy population) by itself to make a diagnosis. (1,2)
  • Accelerated atherosclerosis among these patients is a newly recognized phenomenon responsible for premature mortality. (1,2)
  • Treatment consists primarily of immunosuppressive drugs (e.g., hydroxychloroquine [Plaquenil] and corticosteroids [prednisone]). (1,2) In 2011 the FDA approved the first new drug for lupus in more than 50 years—belimumab [BENLYSTA®].
  • Morbidity and mortality may be related to late diagnosis, problems in access to care, less effective treatments, and poor compliance with therapeutic regimens. (1,2)

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II. Prevalence

  • Prevalence estimates vary widely, and range as high as 1,500,000 (Lupus Foundation of America). (3) A recent study estimated a 2005 prevalence of 161,000 with definite SLE and 322,000 with definite or probable SLE. (8)
  • Congress has funded CDC to conduct two population-based SLE registries with the primary purpose of generating better prevalence (and incidence) estimates for Caucasians and African Americans. One is in Michigan (Washtenaw and Wayne Counties) and the other is in Georgia (DeKalb and Fulton Counties). New registries in California (San Francisco and San Mateo Counties) and New York City (Manhattan) are funded to generate similar estimates for Hispanics and Asians. The Indian Health Service is developing similar estimates for American Indians/Alaska Natives.

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III. Incidence

  • National incidence data are difficult to obtain because onset is difficult to determine (non-specific symptoms and signs) and the required, resource-intense studies are done in small areas. (2)
  • Existing estimates range widely, from 1.8 to 7.6 cases per 100,000 persons per year in parts of the continental United States. (2)
  • Incidence rates in whites in Rochester, Minnesota (Mayo Clinic’s Rochester Epidemiology Project) tripled from 1.5/100,000 in the 1950–1979 cohort to 5.6/100,000 in 1980–1992 cohort. (4)

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IV. Mortality

  • From 1979 to 1998, the annual number of deaths with lupus 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. (5)
  • An equivalent number listed lupus as a contributing cause of death. (5)
  • Causes of death are mainly active disease, organ failure (e.g., kidneys), infection, or cardiovascular disease from accelerated atherosclerosis. (5)
  • Among rheumatic conditions, lupus has a relatively high mortality (14.5% of all rheumatic disease mortality in 1997). (5)
  • At the same time, survival has been improving, suggesting that more or milder cases are being recognized. (5)

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V. Hospitalizations

  • From 1988 through 2000 hospitalizations with lupus listed as a discharge diagnosis increased from <60,000/year to >100,000/year, with an annual average of 77,000 hospitalizations. (6)
  • These patients were younger, more apt to be black or female, had more comorbidities, and had a longer length of stay than patients without lupus. (6)

  • An analysis of 2004 data from the Nationwide Inpatient Sample estimated 13,000 hospitalizations with a principal diagnosis of SLE and 141,000 hospitalizations with principal or secondary diagnosis of lupus. (7)

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VI. Ambulatory Care

  • Lupus was listed for 1,032,000 ambulatory care visits annually from 2001-2005. (9)

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VII. Costs

  • No national cost estimates exist for lupus.

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VIII. Impact on health-related quality of life (HRQOL)

  • No data relating lupus to HRQOL.

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IX. Unique characteristics

  • There have been no new treatments for lupus in the past several decades until the 2011 FDA approval of belimumab.
  • Compliance is 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.
  • Patients with lupus have an increased frequency of related 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.
  • Patients can have symptoms and signs of lupus and scleroderma, called mixed connective tissue disease.
  • Neonatal lupus occurs as a result of passively acquired auto antibodies from a mother with lupus. Skin, liver, and blood problems resolve by 6 months, but the most serious manifestation—congenital heart block—requires a pacemaker and has a mortality rate of ~20%.

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X. References

  1. Pisetsky DS, Buyon JP, Manzi S. Chapter 17. Systemic lupus erythematosus. In: Klippel JH, Crofford LJ, Stone JH, Weyand CM. Primer on the Rheumatic Diseases. Edition 12. Arthritis Foundation, Atlanta, GA., 2001.
  2. Rus V, Hajeer A, Hochberg MC. Chapter 7. Systemic lupus erythematosus. In: Silman AJ, Hochberg MC (eds.) Epidemiology of the Rheumatic Disease. 2nd edition. Oxford University Press, New York, 2001.
  3. Lawrence RC, Felson DT, Helmick CG, Arnold LM, Choi H, Deyo RA, Gabriel S, Hirsch R, Hochberg MC, Hunder GG, Jordan JM, Katz JN, Maradit Kremers H, and Wolfe F for the National Arthritis Data Workgroup. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: Part II. Arthritis Rheum 2008;58(1):26–35. abstract; pdf [309K]
  4. 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.
  5. Sacks JJ, Helmick CG, Langmaid G, Sniezek JE. Trends in deaths from systemic lupus erythematosus—United States, 1979–1998. MMWR 2002;51(17):371–374.
  6. Lethbridge-Cejku M, Helmick CG, Popovic JR. Hospitalizations for systemic lupus erythematosus in the United States: 1988–2000 (unpublished).
  7. United States Bone and Joint Decade: The Burden of Musculoskeletal Diseases in the United States. Rosemont, IL: American Academy of Orthopaedic Surgeons; 2008. Chapter 4. Arthritis and Related Conditions.
  8. Helmick CG, Felson DT, Lawrence RC, Gabriel S, Hirsch R, , Kwoh CK, Liang MH, Maradit Kremers H, Mayes MD, Merkel PA, Pillemer SR, Reveille JD, and Stone JH for the National Arthritis Data Workgroup. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States: Part I. Arthritis Rheum 2008;58(1):15–25.
  9. Sacks JJ, Luo Y-H, Helmick CG. Prevalence of specific types of arthritis and other rheumatic conditions in the ambulatory health care system in the United States, 2001-2005. Arthritis Care & Research 2010;62(4):460-464.

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XI. Resources

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