Systemic lupus erythematosus (SLE) is a long-term autoimmune disorder that may affect the skin, joints, kidneys, brain, and other organs.A.D.A.M.

Causes, incidence, and risk factors

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.

SLE may also be caused by certain drugs. For information on this cause, see Drug-induced lupus erythematosus

Symptoms

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
• Fatigue
• Fever with no other cause
• General discomfort, uneasiness, or ill feeling (malaise)
• Hair loss
• Mouth sores
• 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.
• Swollen lymph nodes

Other symptoms depend on what part of the body is affected:

• Brain and nervous system: headaches, numbness, tingling, seizures, vision problems, personality changes
• Digestive tract: abdominal pain, nausea, and vomiting
• Heart: abnormal heart rhythms (arrhythmias)
• Lung: coughing up blood and difficulty breathing
• Skin: patchy skin color, fingers that change color when cold (Raynaud's phenomenon)

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.

Tests used to diagnose SLE may include:

• Antibody tests, including antinuclear antibody (ANA) panel
• CBC
• Chest x-ray
• Kidney biopsy
• Urinalysis

This disease may also alter the results of the following tests:

• Antithyroglobulin antibody
• Antithyroid microsomal antibody
• Complement components (C3 and C4)
• Coombs' test - direct
• Cryoglobulins
• ESR
• Kidney function blood tests
• Liver function blood tests
• Rheumatoid factor

This list is not all inclusive.

Treatment

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
• Up-to-date immunizations
• 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.

Support Groups

See: Lupus resources

Expectations (prognosis)

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. The disease tends to be more active in the first years after diagnosis and in younger patients (less than 40 years old).

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.

Complications

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:

• Blood clots in the legs (deep vein thrombosis) or lungs (pulmonary embolism)
• Destruction of red blood cells (hemolytic anemia) or anemia of chronic disease
• Fluid around the heart (pericarditis), endocarditis, or inflammation of the heart (myocarditis)
• Fluid around the lungs (pleural effusions) and damage to lung tissue
• Pregnancy complications, including miscarriage
• Stroke
• Severely low blood platelets (thrombocytopenia)
• Inflammation of the blood vessels

Calling your health care provider

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.

References

1. 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. [PubMed: 19103632]
2. 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.

Review Date: 2/2/2012.

Reviewed by: Ariel D. Teitel, MD, MBA, Clinical Associate Professor of Medicine, NYU Langone Medical Center. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.