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Systemic lupus erythematosus

Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus; Butterfly rash - SLE; Discoid lupus

Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It can affect the skin, joints, kidneys, brain, and other organs.

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Systemic lupus erythematosus
Lupus, discoid  - view of lesions on the chest
Lupus - discoid on a child's face
Systemic lupus erythematosus rash on the face
Antibodies

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Causes

The cause of SLE is not clearly known. It may be linked to the following factors:

SLE is more common in women than men by nearly 10 to 1. It may occur at any age. However, it appears most often in young women between the ages of 15 and 44. In the US, the disease is more common in African Americans, Asian Americans, African Caribbeans, and Hispanic Americans.

Symptoms

Symptoms vary from person to person, and may come and go. Everyone with SLE has joint pain and swelling at some time. Some develop arthritis. SLE often affects the joints of the fingers, hands, wrists, and knees.

Other common symptoms include:

Other symptoms and signs depend on which part of the body is affected:

Some people have only skin symptoms. This is called discoid lupus.

Exams and Tests

To be diagnosed with lupus, you must have 4 out of 11 common signs of the disease. Nearly all people with lupus have a positive test for antinuclear antibody (ANA). However, having a positive ANA alone does not mean you have lupus.

The health care provider will do a complete physical exam. You may have a rash, arthritis, or edema in the ankles. There may be an abnormal sound called a heart friction rub or pleural friction rub. Your provider will also do a nervous system exam.

Tests used to diagnose SLE may include:

You may also have other tests to learn more about your condition. Some of these are:

Treatment

There is no cure for SLE. The goal of treatment is to control symptoms. Severe symptoms that involve the heart, lungs, kidneys, and other organs often need treatment by specialists. Each person with SLE needs evaluation regarding:

Mild forms of the disease may be treated with:

Treatments for more severe SLE may include:

If you have SLE, it is also important to:

Support Groups

Counseling and support groups may help with the emotional issues involved with the disease.

Outlook (Prognosis)

The outcome for people with SLE has improved in recent years. Many people with SLE have mild symptoms. How well you do depends on how severe the disease is. Most people with SLE will require medicines for a long time. Nearly all will require hydroxychloroquine indefinitely. However, in the US, SLE is one of the top 20 leading causes of death in females between the ages of 5 and 64. Many new medicines are being studied to improve the outcome of women with SLE.

The disease tends to be more active:

Many women with SLE can get pregnant and deliver a healthy baby. A good outcome is more likely for women who receive proper treatment and do not have serious heart or kidney problems. However, the presence of certain SLE antibodies or antiphospholipid antibodies raises the risk of miscarriage.

Possible Complications

LUPUS NEPHRITIS

Some people with SLE have abnormal immune deposits in the kidney cells. This leads to a condition called lupus nephritis. People with this problem may develop kidney failure. They may need dialysis or a kidney transplant.

A kidney biopsy is done to detect the extent of damage to the kidney and to help guide treatment. If active nephritis is present, treatment with immunosuppressive medicines including high doses of corticosteroids along with either cyclophosphamide or mycophenolate are needed.

OTHER PARTS OF THE BODY

SLE can cause damage in many different parts of the body, including:

SLE AND PREGNANCY

Both SLE and some of the medicines used for SLE can harm an unborn child. Talk to your provider before you become pregnant. If you become pregnant, find a provider who is experienced with lupus and pregnancy.

When to Contact a Medical Professional

Call your provider if you have symptoms of SLE. Also call if you have this disease and your symptoms get worse or a new symptom occurs.

Related Information

Autoimmune disorders
Antibody
Drug-induced lupus erythematosus
Joint pain
Arthritis
Rashes
Skin nodules
Lupus nephritis
Acute kidney failure
Confusion
Seizures
Psychosis
Neurocognitive disorder
Headache
Blood clots
Pulmonary embolus
Antiphospholipid syndrome - APS
Platelet count
Pericarditis
Endocarditis
Myocarditis
Chest pain
Heart palpitations
Pleurisy
Pleural effusion
Breathing difficulty
Thrombocytopenia
Hemolytic anemia

References

Arntfield RT, Hicks CM. Systemic lupus erythematosus and the vasculitides. In: Walls RM, Hockberger RS, Gausche-Hill M, eds. Rosen's Emergency Medicine: Concepts and Clinical Practice. 9th ed. Philadelphia, PA: Elsevier; 2018:chap 108.

Crow MK. Etiology and pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 79.

Fanouriakis A, Kostopoulou M, Alunno A, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6) :736-745. PMID: 30926722 pubmed.ncbi.nlm.nih.gov/30926722/.

Hahn BH, McMahon MA, Wilkinson A, et al. American College of Rheumatology guidelines for screening, treatment, and management of lupus nephritis. Arthritis Care Res (Hoboken). 2012;64(6):797-808. PMID: 22556106 pubmed.ncbi.nlm.nih.gov/22556106/.

van Vollenhoven RF, Mosca M, Bertsias G, et al. Treat-to-target in systemic lupus erythematosus: recommendations from an international task force. Ann Rheum Dis. 2014;73(6):958-967. PMID: 24739325 pubmed.ncbi.nlm.nih.gov/24739325/.

Yen EY, Singh RR. Brief Report: lupus - an unrecognized leading cause of death in young females: a population-based study using nationwide death certificates, 2000-2015. Arthritis Rheumatol. 2018;70(8):1251-1255. PMID: 29671279 pubmed.ncbi.nlm.nih.gov/29671279/.

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Review Date: 1/21/2020  

Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. Editorial update 09/30/2020.

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