Systemic lupus erythematosus

Definition

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.

Alternative Names

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

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

The American and European Rheumatology societies have published classification criteria to assist the diagnosis of SLE. These include specific symptoms, physical finding and laboratory tests. Nearly all people with SLE have a positive test for antinuclear antibody (ANA). However, having a positive ANA alone does not mean you have SLE.

The health care provider will do a complete physical exam. You may have a rash, arthritis, or swelling 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 United States, 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 SLE and pregnancy.

When to Contact a Medical Professional

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

References

Aringer M, Costenbader K, Daikh D, et al. 2019 European League against rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Arthritis Rheumatol. 2019;71(9):1400-1412. PMID: 31385462 pubmed.ncbi.nlm.nih.gov/31385462/.

Crow MK. Etiology and pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 84.

Crow MK. Systemic lupus erythematosus. In: Goldman L, Cooney KA, eds. Goldman-Cecil Medicine. 27th ed. Philadelphia, PA: Elsevier; 2024:chap 245. 

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/.


Review Date: 1/25/2023
Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Internal review and update on 01/25/2024 by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
© 1997- adam.comAll rights reserved.