Systemic lupus erythematosus (SLE) is an autoimmune disease, which means the body's immune system mistakenly attacks healthy tissue. This leads to long-term (chronic) inflammation.
The underlying cause of autoimmune diseases is not fully known.
SLE is much more common in women than men. It may occur at any age, but appears most often in people between the ages of 10 and 50. African Americans and Asians are affected more often than people from other races.
Symptoms vary from person to person, and may come and go. Almost everyone with SLE has joint pain and swelling. Some develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees.
Other common symptoms include:
Chest pain when taking a deep breath
Fever with no other cause
General discomfort, uneasiness, or ill feeling (malaise)
Sensitivity to sunlight
Skin rash -- a "butterfly" rash over the cheeks and bridge of the nose affects about half of people with SLE. The rash gets worse in sunlight. The rash may also be widespread.
Some patients only have skin symptoms. This is called discoid lupus.
Signs and tests
To be diagnosed with lupus, you must have 4 out of 11 typical signs of the disease.
Your doctor will perform a physical exam and listen to your chest with a stethoscope. An abnormal sound called a heart friction rub or pleural friction rub may be heard. A nervous system exam will also be done.
There is no cure for SLE. The goal of treatment is to control symptoms.
Mild disease may be treated with:
Nonsteroidal anti-inflammatory medications (NSAIDs) treat arthritis and pleurisy
Corticosteroid creams to treat skin rashes
An antimalaria drug (hydroxychloroquine) and low-dose corticosteroids for skin and arthritis symptoms
You should wear protective clothing, sunglasses, and sunscreen when in the sun.
Severe or life-threatening symptoms (such as hemolytic anemia, extensive heart or lung involvement, kidney disease, or central nervous system involvement) often require more aggressive treatment by doctor specialists.
Treatment for more severe lupus may include:
High-dose corticosteroids or medications to decrease the immune system response
Cytotoxic drugs (drugs that block cell growth) if you do not get better with corticosteroids, or whose symptoms get worse when the stop taking them. These medicine have serious, severe side effects. You should be closely monitored by your doctor.
If you have lupus, it is also important to have:
Preventive heart care
Tests to screen for thinning of the bones (osteoporosis)
Talk therapy and support groups may help relieve depression and mood changes that may occur in patients with this disease.
How well a person does depends on the severity of the disease.
The outcome for people with SLE has improved in recent years. Many people with SLE have mild symptoms.
Women with SLE who become pregnant are often able to carry safely to term and deliver a healthy infant, as long as they do not have severe kidney or heart disease and the SLE is being treated appropriately. However, the presence of SLE antibodies may increase the risk of pregnancy loss.
Some people with SLE have abnormal deposits in the kidney cells. This leads to a condition called lupus nephritis. Patients with this condition may eventually develop kidney failure and need dialysis or a kidney transplant.
SLE causes damage to many different parts of the body, including:
Call your health care provider if you have symptoms of SLE. Also, call if you have this disease and your symptoms get worse or a new one occurs.
Ruiz-Irastorza G, Ramos-Casals M, Brito-Zeron P, Khamashta MA. Clinical efficacy and side effects of antimalarials in systemic lupus erythematosus: a systematic review. Ann Rheum Dis. 2010;69:20-28.
Hahn BH, Tsao BP. Pathogenesis of systemic lupus erythematosus. In: Firestein GS, Budd RC, Harris ED Jr., et al., eds. Kelley's Textbook of Rheumatology. 8th ed. Philadelphia, Pa: Saunders Elsevier; 2008:chap 74.
Michael E. Makover, MD is a professor and attending in rheumatology at the New York University Medical Center, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.