Systemic lupus erythematosus

Also known as: Disseminated lupus erythematosus, SLE, Lupus or Lupus erythematosus

Definition

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

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.

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.

See also:

• Disease

  Organic brain syndrome

  

  Organic brain syndrome (OBS) is a general term used to describe decreased mental function due to a medical disease, other than a psychiatric illness. It is often used synonymously (but incorrectly) with .

• Disease

  Thrombocytopenia

  Thrombocytopenia is any disorder in which there is an abnormally low amount of platelets. Platelets are parts of the blood that help blood to clot. This condition is sometimes associated with abnormal bleeding.

• Disease

  Acute kidney failure

  

  Acute (sudden) kidney failure is the sudden loss of the ability of the kidneys to remove waste and concentrate urine without losing .

• Special Topic

  Antibody

  

  An antibody is a protein produced by the body’s immune system when it detects harmful substances, called antigens. Examples of antigens include microorganisms (such as bacteria, fungi, parasites, and viruses) and chemicals. Antibodies are also produced when the immune system mistakenly ...

• Symptoms

  Heart palpitations

  

  Palpitations are heartbeat sensations that feel like your heart is pounding or racing. You may simply have an unpleasant awareness of your own heartbeat, or may feel skipped or stopped beats. The heart’s rhythm may be normal or abnormal. Palpitations can be felt in your chest, throat, or ...

• Disease

  Pleurisy

  

  Pleurisy is inflammation of the lining of the lungs and chest (the pleura) that leads to chest pain (usually sharp) when you take a breath or cough.

• Symptoms

  Chest pain

  Chest pain is discomfort or pain that you feel anywhere along the front of your body between your neck and upper abdomen.

• Disease

  Endocarditis

  

  Endocarditis is inflammation of the inside lining of the heart chambers and heart valves (endocardium). See also:

• Symptoms

  Nodules

  

  Nodules are solid, raised bumps in or under the skin that are wider than 10 millimeters.

• Disease

  Autoimmune disorders

  

  An autoimmune disorder is a condition that occurs when the immune system mistakenly attacks and destroys healthy body tissue. There are more than 80 different types of autoimmune disorders. See also:

• Disease

  Pleural effusion

  

  A pleural effusion is a buildup of fluid between the layers of tissue that line the lungs and chest cavity.

• Disease

  Pulmonary embolus

  

  A pulmonary embolus is a blockage of an artery in the lungs by fat, air, a blood clot, or tumor cells.

• Test

  Dialysis

  

  Dialysis is a method of removing toxic substances (impurities or wastes) from the blood when the kidneys are unable to do so.

• Symptoms

  Joint pain

  

  Joint pain can affect one or more joints. See also: (inflammation of joints)

• Disease

  Myocarditis

  

  Myocarditis is inflammation of the heart muscle. See also:

• Symptoms

  Seizures

  A seizure is the physical findings or changes in behavior that occur after an episode of abnormal electrical activity in the brain. See also: (seizures in children with high fever)

• Symptoms

  Headache

  

  A headache is pain or discomfort in the head, scalp, or neck. Serious causes of headaches are very rare. Most people with headaches can feel much better by making lifestyle changes, learning ways to relax, and sometimes by taking medications. See also:

• Symptoms

  Confusion

  

  Confusion is the inability to think with your usual speed or clarity, including feeling disoriented and having difficulty paying attention, remembering, and making decisions. See also: , for more information about chronic or long-term confusion.

• Disease

  Blood clots

  

  Blood clots are clumps that occur when blood hardens from a liquid to a solid. A blood clot that forms inside a blood vessel or within the heart and stays there is called a thrombus. A thrombus that breaks loose and travels from one location in the body to another is called an embolus. The ...

• Disease

  Lupus anticoagulants

  

  Lupus anticoagulants are antibodies against phospholipids (substances in the lining of cells) that prevent blood clotting in a test tube. Persons with these antibodies may have an abnormally high risk of blood clotting. See also:

• Disease

  Psychosis

  Psychosis is a loss of contact with reality, usually including false beliefs about what is taking place or who one is (delusions) and seeing or hearing things that aren’t there (hallucinations).

• Disease

  Arthritis

  

  Arthritis is inflammation of one or more joints, which results in pain, swelling, stiffness, and limited movement. There are over 100 different types of arthritis. See also:

• Disease

  Lupus nephritis

  

  Lupus nephritis is a kidney disorder that is a complication of .

• Disease

  Drug-induced lupus erythematosus

  

  Drug-induced lupus erythematosus is an that is brought on by a reaction to medication. See also:

• Test

  Platelet count

  A platelet count is a test to measure how many platelets you have in your blood. Platelets help the blood clot. They are smaller than red or white blood cells.

• Symptoms

  Rashes

  

  Rashes involve changes in the color or texture of your skin.

• Symptoms

  Breathing difficulty

  

  Breathing difficulty involves a sensation of difficult or uncomfortable breathing or a feeling of not getting enough air. See also:

• Disease

  Hemolytic anemia

  

  Hemolytic is a condition in which there are not enough red blood cells in the blood, due to the premature destruction of red blood cells. There are a number of specific types of hemolytic anemia, which are described individually.

• Disease

  Pericarditis

  

  Pericarditis is a condition in which the sac-like covering around the heart (pericardium) becomes inflamed. See also:

Review date:

February 7, 2010

Reviewed by:

Mark James Borigini, MD, Rheumatologist in the Washington, DC Metro area. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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