Systemic lupus erythematosus

Definition

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder. SLE may affect the skin, joints, kidneys, and other organs.

Alternative Names

Disseminated lupus erythematosus; SLE; Lupus; Lupus erythematosus

Causes, incidence, and risk factors

SLE (lupus) is an autoimmune disease. This means there is a problem with the body's normal immune system response.

Normally, the immune system helps protect the body from harmful substances. But in patients with an autoimmune disease, the immune system cannot tell the difference between harmful substances and healthy ones. The result is an overactive immune response that attacks otherwise healthy cells and tissue. This leads to long-term (chronic) inflammation.

The underlying cause of autoimmune diseases is not fully known.

SLE may be mild or severe enough to cause death.

SLE affects nine times as many women as 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 of SLE, see drug-induced lupus erythematosus.

Symptoms

Symptoms vary from person to person, and may come and go. The condition may affect one organ or body system first. Others may become involved later.

Almost all people with SLE have 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
  • Mild cognitive impairment
  • Numbness, tingling, or pain in the arms or legs
  • Personality change
  • Psychosis
  • Risk of stroke
  • Seizures
  • Vision problems
• Digestive tract: abdominal pain, nausea, and vomiting
• Heart: abnormal heart rhythms (arrhythmias)
• Kidney: blood in the urine
• Lung: coughing up blood and difficulty breathing
• Skin: patchy skin color, fingers that change color when cold (Raynaud's phenomenon)

Signs and tests

The diagnosis of SLE is based upon the presence of at least 4 out of 11 typical characteristics of the disease. The doctor will listen to your chest with a stethoscope. A sound called a heart friction rub or pleural friction rub may be heard. A neurological exam will also be performed.

Tests used to diagnose SLE may include:

• Antibody tests, including:
  • Antinuclear antibody (ANA) panel
  • Anti-double strand (ds) DNA
  • Antiphospholipid antibodies
  • Anti-Smith antibodies
• CBC to show low white blood cells, hemoglobin, or platelets
• Chest x-ray showing pleuritis or pericarditis
• Kidney biopsy
• Urinalysis to show blood, casts, or protein in the urine

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

• Anti-SSA or -SSB antibodies
• Antithyroglobulin antibody
• Antithyroid microsomal antibody
• Complement components (C3 and C4)
• Coombs' test - direct
• Cryoglobulins
• ESR
• Rheumatoid factor
• RPR - a test for syphilis
• Serum globulin electrophoresis
• Serum protein electrophoresis

Treatment

There is no cure for SLE. Treatment is aimed at controlling symptoms. Your individual symptoms determine your treatment.

Mild disease that involves a rash, headaches, fever, arthritis, pleurisy, and pericarditis does not need much therapy.

• Nonsteroidal anti-inflammatory medications (NSAIDs) are used to treat arthritis and pleurisy.
• Corticosteroid creams are used to treat skin rashes.
• An antimalaria drug (hydroxychloroquine) and low-dose corticosteroids are sometimes used 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 treatment by a rheumatologist and other specialists.

• Corticosteroids or medications to decrease the immune system response may be prescribed to control the various symptoms.
• Cytotoxic drugs (drugs that block cell growth) are used to treat people who do not respond well to corticosteroids, or who are unable to stop taking corticosteroids without their symptoms getting worse.

Support Groups

For additional information and support, see lupus resources.

Expectations (prognosis)

The outcome for people with SLE has improved in recent years. Many people with SLE have mild illness. Women with SLE who become pregnant are often able to carry safely to term and deliver a normal infant, as long as they do not have severe kidney or heart disease and the SLE is being treated appropriately.

The presence of antiphospholipid antibodies may increase the possibility of pregnancy loss.

The 10-year survival rate for lupus patients is greater than 85%. People with severe involvement of the brain, lungs, heart, and kidney do worse than others in terms of overall survival and disability.

Complications

Some people with SLE have deposits of antibodies in the cells (glomeruli) of the kidneys. 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), damage to the lung tissue (interstitial lung disease)
• Pregnancy complications, including miscarriage and flare-up of SLA during pregnancy
• Stroke
• Severely low blood platelets (thrombocytopenia)
• Vasculitis, which may damage arteries anywhere in the body

Calling your health care provider

Call your health care provider if you develop symptoms of SLE. Also, call if you have SLE and symptoms get worse or if new symptoms develop.

Prevention

visHeader

References

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.