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Lupus Detailed Fact Sheet

This detailed fact sheet is an overview of the epidemiology of systemic lupus erythematosus (SLE). For more basic information for individuals with general questions about lupus, please visit the lupus basic fact sheet.

Lupus

Lupus is an autoimmune disease that triggers inflammation in different tissues of the body. Autoimmune diseases happen when the body’s immune system attacks its own tissues. The most common type of lupus is systemic lupus erythematosus (SLE), which affects different parts of the body including internal organs.

Systemic Lupus Erythematosus (SLE)

What is SLE?

SLE, a type of lupus, is an autoimmune disease in which the immune system attacks its own tissues. It can affect the joints, skin, brain, lungs, kidneys, and blood vessels, and can cause widespread inflammation and tissue damage in the affected organs. There is no cure for lupus, but medical interventions and lifestyle changes can help control it.

How serious is SLE?

The seriousness of SLE can range from mild to life-threatening. The disease should be treated by a doctor or a team of doctors who specialize in care of SLE patients. People with lupus that get proper medical care, preventive care, and education can significantly improve function and quality of life.

What causes SLE?

The causes of SLE are unknown, but are believed to be linked to environmental, genetic, and hormonal factors.

What are the signs and symptoms? 

People with SLE may experience a variety of symptoms that include fatigue, skin rashes, fevers, and pain or swelling in the joints. SLE symptoms may affect women and men differently. Among some adults, having a period of SLE symptoms—called flares—may happen every so often, sometimes even years apart, and go away at other times—called remission. However, other adults may experience SLE flares more frequently throughout their life.

Other symptoms can include sun sensitivity, oral ulcers, arthritis, lung problems, heart problems, kidney problems, seizures, psychosis, and blood cell and immunological abnormalities.

What are the complications of SLE? 

SLE can have both short- and long-term effects on a person’s life. Early diagnosis and effective treatments can help reduce the damaging effects of SLE and improve the chance to have better function and quality of life. Poor access to care, late diagnosis, less effective treatments, and poor adherence to therapeutic regimens may increase the damaging effects of SLE, causing more complications and an increased risk of death.1

SLE can limit a person’s physical, mental, and social functioning. These limitations experienced by women and men with SLE can impact their quality of life, especially if they experience fatigue. Fatigue is the most common symptom affecting the quality of life of people with SLE.2,3

Many studies use employment as a measure to determine the quality of life of people with SLE as employment is central to a person’s life.3 Some studies have shown that the longer a person has had SLE, the less likely they are to be a part of the workforce. On average only 46% of people with SLE of working age report being employed.3

Adherence to treatment regimens is often a problem, especially among young, reproductive age women. Because SLE treatment may require the use of strong immunosuppressive medications that can have serious side effects, female patients must stop taking the medication before and during pregnancy to protect unborn children from harm.

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SLE Diagnosis

How is SLE diagnosed? 

SLE is diagnosed by a health care provider using symptom assessments, physical examination, X-rays, and lab tests. SLE may be difficult to diagnose because its signs and symptoms are not specific and can look like signs and symptoms of other diseases.1 SLE may also be misdiagnosed if only a blood test is used for diagnosis. Because diagnosis can be challenging, it is important to see a doctor specializing in rheumatology for a final diagnosis. Rheumatologists sometimes use specific criteria [PDF -510KB] for diagnosing SLE.

Who is at risk for SLE? 

SLE can affect people of all ages, including children. However, women of childbearing ages—15 to 44 years—are at greatest risk of developing SLE.1  Women are affected far more than men (estimates range from 4 to 12 women for every 1 man).1

Minority and ethnic groups—blacks/African Americans, Hispanics/Latinos, Asians, and American Indians/Alaska Natives—are affected more than whites.1

Does SLE Run in Families? 

Most people with SLE do not have family members with the disease; however, some people with SLE do have a family history of the disease. Men and women with an immediate family member with SLE have only a slightly higher risk for the disease.

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SLE Treatment

How is SLE treated? 

A team approach in treating SLE is often needed because of the number of organs that can be involved.

SLE treatment consists primarily of immunosuppressive drugs that inhibit activity of the immune system. Hydroxychloroquine and corticosteroids (e.g., prednisone) are often used to treat SLE. The FDA approved belimumab in 2011, the first new drug for SLE in more than 50 years.

SLE also may occur with other autoimmune conditions that require additional treatments, like Sjogren’s syndrome, antiphospholipid syndrome, thyroiditis, hemolytic anemia, and idiopathic thrombocytopenia purpura.1

SLE Prevalence and Incidence

Prevalence is a measurement of all individuals affected by the disease at a particular time. Incidence is a measurement of the number of new cases of individuals who contract a disease during a particular period of time, often a year.

Recent national estimates of prevalence and incidence are not available. SLE is relatively uncommon and not a reportable disease, so it is relatively expensive to capture all diagnosed cases reliably for epidemiologic studies. There are no recent studies to determine if SLE prevalence or incidence are changing over time.

CDC funded several population-based patient registries to generate better estimates of the number of cases of diagnosed SLE in certain racial/ethnic groups. The most recent available prevalence and incidence estimates for SLE for whites and blacks, published in 2014, come from these lupus registries. The CDC-funded lupus registries used similar intensive methods for case finding (hospitals, specialists’ practices, health department data) and for seeing if possible cases met standard classification criteria (i.e., medical record review). See the Lupus Studies page for more information.

SLE Prevalence

Older national prevalence estimates vary widely due to differences in case definitions, small study populations, and study methods. A conservative estimate suggests a prevalence of 161,000 with definite SLE and 322,000 with definite or probable SLE.4

Results from the CDC Lupus registries estimated 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 people)5 and in Georgia (DeKalb and Fulton Counties) (128.0 vs 39.9 per100,000 people).6 Annual prevalence from 2007–2009 for American Indians/Alaska Natives was 178 per 100,000.7 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 people),5 in Georgia (145.8 vs 17.5 per 100,000 people),6 and in the American Indian/Alaska Native population (271 vs 54 per 100,000 people).7

SLE Incidence

Recent national incidence estimates are not available for SLE. National incidence data are difficult to obtain because it is relatively expensive to capture all diagnosed cases reliably (learn more about SLE Prevalence and Incidence) and the year of onset is hard to determine (slowly developing, non-specific symptoms and signs), so resource-intense studies must be done in small areas.1 

SLE incidence estimates are available from the various CDC-funded lupus registries. Annual incidence for different racial/ethnic groups from 2002–2004 was much higher for blacks than whites in Michigan (7.9 vs 3.7 100,000 people)5 and in Georgia (9.4 vs 3.2 per 100,000 people).6 Annual incidence from 2007–2009 for American Indians/Alaska Natives was 7.4 per 100,000)7 New registries have begun in California (San Francisco County) and New York City (Manhattan), and 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),5 Georgia (10.6 vs 1.9 per 100,000)6 and the American Indian/Alaska Native population (unadjusted 8.4 vs 2.7 per 100,000).7

Mortality

Causes of premature death associated with SLE are mainly active disease, organ failure (e.g., kidneys), infection, or cardiovascular disease from accelerated atherosclerosis.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 of SLE-associated mortality.9

Using death certificates for US residents, SLE was identified as the underlying cause of death for an average of 1,034 deaths from 2010–2014. SLE was identified as a contributing cause of death (one of multiple causes of death, including underlying cause of death) for an average of 1,803 deaths during that 4-year-period.10

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CDC Activities

What is CDC doing? 

CDC has previously funded five lupus registries and the development of a public health agenda to guide public health efforts. Currently, CDC is funding work on several SLE-relevant activities, such as three follow-up studies and research for self-management. For more information, visit the CDC- funded activities page.

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Other Types of Lupus

SLE is the most common and most serious type of lupus. Other types of lupus include the following:

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. 

Drug-induced lupus is similar to SLE, but occurs as the result of an overreaction to certain medications. Symptoms usually occur 3 to 6 months after starting a medication, and disappear once the medicine is stopped.11 Learn more about Drug-Induced Lupus on the Medline Plus website.

Neonatal lupus occurs when an infant passively acquires 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%.12

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Additional Information

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References

  1. Dall’Era M.  Systemic lupus erythematosus. In: Imboden JB, Hellman DB, Stone JH. (Eds). Current Rheumatology Diagnosis and Treatment. 3rd ed. New York, NY:McGraw-Hill; 2013.
  2. Jolly M, Pickard SA, Mikolaitis RA, Rodby RA, Sequeira W, Block JA. Lupus QoL-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
  3. Yazdany J, 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
  4. 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
  5. 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
  6. 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
  7. 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
  8. 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. 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. Centers for Disease Control and Prevention, National Center for Health Statistics. Compressed Mortality File 1999–2014 on CDC WONDER Online Database. http://wonder.cdc.gov/cmf-icd10.html. Accessed January 27, 2017.
  11. National Library of Medicine. Medline Plus: Drug-Induced Lupus Erythematosus Website. http://www.nlm.nih.gov/medlineplus/ency/article/000446.htm. Accessed January 27, 2017.
  12. 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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