Systemic lupus erythematosus

Systemic Lupus Erythematosus 

Definition

Systemic lupus erythematosus (also called lupus or SLE) is a disease where a person's immune system attacks and injures the body's own organs and tissues. Almost every system of the body can be affected by SLE.

Description

The body's immune system is a network of cells and tissues responsible for clearing the body of invading foreign organisms, like bacteria, viruses, and fungi. Antibodies are special immune cells that recognize these foreign invaders, and begin a chain of events to destroy them. In an autoimmune disorder like SLE, a person's antibodies begin to recognize the body's own tissues as foreign. Cells and chemicals of the immune system damage the tissues of the body. The reaction that occurs in tissue is called inflammation. Inflammation includes swelling, redness, increased blood flow, and tissue destruction.

In SLE, some of the common antibodies that normally fight diseases are thought to be out of control. These include antinuclear antibodies and antiDNA antibodies. Antinuclear antibodies are directed against the cell's central structure that contains genetic material (the nucleus). Anti-DNA antibodies are directed against the cell's genetic material. DNA is the chemical substance that makes up the chromosomes and genes.

SLE can occur in both males and females of all ages, but 90% of patients are women. The majority of these women are in their childbearing years. African Americans are more likely than Caucasians to develop SLE.

Occasionally, medications can cause a syndrome of symptoms very similar to SLE. This is called drug-induced lupus. Medications that may cause this syndrome include hydralazine (used for high blood pressure) and procainamide (used for abnormal heartbeats). Drug-induced lupus almost always disappears after the patient stops taking the medications that caused it.

Causes and symptoms

The cause of SLE is unknown. Because the vast majority of patients are women, some research is being done to determine what (if any) link the disease has to female hormones. SLE may have a genetic basis, although more than one gene is believed to be involved in the development of the disease. Because patients with the disease may suddenly have worse symptoms (called a flare) after exposure to things like sunlight, alfalfa sprouts, and certain medications, researchers suspect that some environmental factors may also be at work.

The severity of a patient's SLE varies over time. Patients may have periods with mild or no symptoms, followed by a flare. During a flare, symptoms increase in severity and new organ systems may become affected.

Many SLE patients have fevers, fatigue, muscle pain, weakness, decreased appetite, and weight loss. The spleen and lymph nodes are often swollen and enlarged. The development of other symptoms in SLE varies, depending on the organs affected.

Diagnosis

Diagnosis of SLE can be somewhat difficult. There are no definitive tests for diagnosing SLE. Many of the symptoms and laboratory test results of SLE patients are similar to those of patients with different diseases, including rheumatoid arthritis, multiple sclerosis, and various nervous system and blood disorders.

Laboratory tests that are helpful in diagnosing SLE include several tests for a variety of antibodies commonly elevated in SLE patients (including antinuclear antibodies, anti-DNA antibodies, etc.). SLE patients tend to have low numbers of red blood cells (anemia) and low numbers of certain types of white blood cells. The erythrocyte sedimentation rate (ESR), a measure of inflammation in the body, tends to be quite elevated. Samples of tissue (biopsies) from affected skin and kidneys show characteristics of the disease.

A test called the lupus erythematosus cell preparation (or LE prep) test is also performed. This test involves obtaining a sample of the patient's blood. Cells from the blood are damaged in the laboratory in order to harvest their nuclei. These damaged cells are then put together with the patient's blood serum, the liquid part of blood separated from the blood cells. Antinuclear antibodies within the patient's serum will clump together with the damaged nuclear material. A material called Wright's stain will cause these clumps to turn blue. These stained clumps are then reacted with some of the patient's white blood cells, which will essentially eat the clumps. LE cells are the white blood cells that contain the blue clumps. This test will be positive in about 70-80% of all patients with SLE.

The American Rheumatism Association developed a list of symptoms used to diagnose SLE. Research supports the idea that people who have at least four of the eleven criteria (not necessarily simultaneously) are extremely likely to have SLE. The criteria are:

Systemic lupus erythematosus (SLE) is an autoimmune disease in which the individual's immune system attacks, injures, and destroys the body's own organs and tissues. Nearly every system of the body can be affected by SLE, as depicted in the illustration above.

(Illustration by Electronic Illustrators Group.)

Treatment

Treatment depends on the organ systems affected by SLE and the severity of the disease. Some patients have a mild form of SLE. Their mild symptoms of inflammation can be treated with nonsteroidal anti-inflammatory drugs like ibuprofen (Motrin, Advil) and aspirin. Severe skin rashes and joint problems may respond to a group of medications usually used to treat malaria. More severely ill patients with potentially life-threatening complications (including kidney disease, pericarditis, or nervous system complications) will require treatment with more potent drugs, including steroid medications. Because steroids have serious side effects, they are reserved for more severe cases of SLE. Drugs that decrease the activity of the immune system (called immunosuppressant drugs) may also be used for severely ill SLE patients. These include azathioprine and cyclophosphamide.

Other treatments for SLE try to help specific symptoms. Clotting disorders will require blood thinners. Psychotic disorders will require specific medications. Kidney failure may require the blood to be cleaned outside the body through a machine (dialysis) or even a kidney transplantation.

Alternative treatment

A number of alternative treatments have been suggested to help reduce the symptoms of SLE. These include acupuncture and massage for relieving the pain of sore joints and muscles. Stress management is key for people with SLE and such techniques as meditation, hynotherapy, and yoga may be helpful in promoting relaxation. Dietary suggestions include eating a whole foods diet with reduced amounts of red meat and dairy products in order to decrease pain and inflammation. Food allergies are believed either to contribute to SLE or to arise as a consequence of the digestive difficulties. Wheat, dairy products, and soy are the major offenders. An elimination/challenge diet can help identify the offending foods so that they can be avoided. Another dietary measure that may be beneficial is eating more fish that contain omega-3 fatty acids, like mackerel, sardines, and salmon. Because alfalfa sprouts have been associated with the onset of flares in SLE, they should be avoided. Supplements that have been suggested to improve the health of SLE patients include vitamins B, C, and E, as well as selenium, zinc, magnesium, and a complete trace mineral supplement. Vitamin A is believed to help improve discoid skin rashes. Botanical medicine can help the entire body through immune modulation and detoxification, as well as assisting individual organs and systems. Homeopathy and flower essences can work deeply on the emotional level to help people with this difficult disease.

Prognosis

The prognosis for patients with SLE varies, depending on the organ systems most affected and the severity of inflammation. Some patients have long periods of time with mild or no symptoms. About 90-95% of patients are still living after 2 years with the disease. About 82-90% of patients are still living after 5 years with the disease. After 10 years, 71-80% of patients are still alive, and 63-75% are still alive after 20 years. The most likely causes of death during the first 10 years include infections and kidney failure. During years 11-20 of the disease, the most likely cause of death involves the development of abnormal blood clots.

Because SLE frequently affects women of childbearing age, pregnancy is an important issue. For pregnant SLE patients, about 30% of the pregnancies end in miscarriage. About 25% of all babies born to mothers with SLE are premature. Most babies born to mothers with SLE are normal. However, a rare condition called neonatal lupus causes a baby of a mother with SLE to develop a skin rash, liver or blood problems, and a serious heart condition.

Prevention

There are no known ways to avoid developing SLE. However, it is possible for a patient who has been diagnosed with SLE to prevent flares of the disease. Recommendations for improving general health to avoid flares include decreasing sun exposure, getting sufficient sleep, eating a healthy diet, decreasing stress, and exercising regularly. It is important for a patient to try to identify the early signs of a flare (like fever, increased fatigue, rash, headache). Some people believe that noticing and responding to these warning signs will allow a patient with SLE to prevent a flare, or at least to decrease its severity.

Key terms

Autoimmune disorder — A disorder in which the body's antibodies mistake the body's own tissues for foreign invaders. The immune system then attacks and causes damage to these tissues.

Chromosomes — Spaghetti-like structures located within the nucleus (or central portion) of each cell. Chromosomes contain genes, structures that direct the growth and functioning of all the cells and systems in the body. Chromosomes are responsible for passing on hereditary traits from parents to child.

Immune system — The system of specialized organs, lymph nodes, and blood cells throughout the body that work together to prevent foreign organisms (bacteria, viruses, fungi, etc.) from invading the body.

Psychosis — Extremely disordered thinking with a poor sense of reality; may include hallucinations (seeing, hearing, or smelling things that are not really there).

Resources

Organizations

American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345. (404) 633-3777. http://www.rheumatology.org.

Lupus Foundation of America. 1300 Piccard Dr., Suite 200, Rockville, MD 20850. (800) 558-0121. http://www.lupus.org.

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systemic lupus er·y·the·ma·to·sus (r-thm-tss, -thm-)

n.

Abbr. SLE A chronic multisystemic inflammatory disease with variable features, frequently including fever, weakness and fatigability, joint pains or arthritis, skin lesions on the face, neck, or upper extremities, and often affecting the kidneys, spleen, and various other organs. Also called disseminated lupus erythematosus.

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systemic lupus erythematosus (SLE) (si·steˑ·mik lōōˑ·ps erˈ··thēˈ·m·tōˑ·sis),

n an autoimmune condition characterized by mouth ulcers, ruddy butterfly rash across the face, and damage to the valves of the heart.

Systemic lupus erythematosus (SLE).

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systemic lupus erythematosus (SLE) (sistem´ik loo´pus er´-them´tō´sus),

n an autoimmune disease that can be life threatening. Patients may have a distinctive pattern of facial redness and oral lesions. Endothelial damage to heart valves similar to that caused by rheumatic fever may occur.