Systemic lupus erythematosus

Systemic lupus erythematosus (also called lupus, or SLE) is a disease in which 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.

The body's immune system is a network of cells and tissues responsible for fighting off invading foreign organisms such as bacteria, viruses, and fungi. Antibodies are special immune cells that recognize these foreign invaders, andbegin 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,causing inflammation of the tissues. In SLE, some of the common antibodies that normally fight diseases are thought to be out of control, and begin to attack the cell's central structure that contains genetic material.

SLE can occur in both men and women of all ages, but 90% of patients are women and most are in their childbearing years. African Americans are more likelythan Caucasians to develop SLE.

Occasionally, medications can cause a syndrome of symptoms very similar to SLE called drug-induced lupus. Medications that may cause this syndrome includehydralazine (used to treat 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.

The cause of SLE is unknown, but it is likely there are many factors that influence its development. Because the vast majority of patients are women, scientists suspect the disease may be associated with female hormones. SLE also 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 get worse after exposure to a range of substances such as sunlight, alfalfa sprouts, and certain medications, researchers suspect that some environmental factors may also be at work.

The severity of a patient's condition changes over time. Patients may have periods with mild or no symptoms, followed by a sudden worsening as new organsbecome affected. Many SLE patients have fevers, fatigue, muscle pain, weakness, loss of 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.

  • Joints. About 90% of SLE patients have jointpain and problems such as arthritis.
  • Skin. A number of skin rashes may occur, including a red butterfly-shaped rash that spreads across the noseand cheekbones or a coin-shaped rash of red, scaly bumps on the cheeks, nose,scalp, ears, chest, back, and the tops of the arms and legs. The roof of themouth may develop sore, irritated ulcers. Hair loss is common. SLE patientstend to be very sensitive to the sun.
  • Lungs. Inflammation of the lungs and tissues of the chest cavity causes a fluid build up in the lungs, withcoughing and shortness of breath.
  • Heart and circulatory system. The tissue surrounding the heart or the heart itself may become inflamed. These heart problems may result in abnormal beats (arrhythmias), problems in pumpingthe blood strongly enough or even sudden death. Blood clots often form in theblood vessels and may lead to complications.
  • Nervous system. Headaches, seizures, personality changes, and confusion may occur.
  • Kidneys.The kidneys may be damaged so that they can't filter the blood, which couldlead to kidney failure.
  • Gastrointestinal system. Patients may experience nausea, vomiting, diarrhea, and abdominal pain. The lining of the abdomen may become inflamed (peritonitis).
  • Eyes. The eyes may become red, sore, and dry. Inflammation of the nerves responsible for eyesight may cause vision problems; inflammation of the blood vessels that serve the retina may lead to blindness.

Diagnosis of SLE is not easy, since there are no sure-fire tests for the condition. Many of the symptoms and test results of SLE patients are similar to those of patients with different diseases, including rheumatoid arthritis, multiple sclerosis, and various other nervous system and blood disorders.

Tests may check for a high level of certain antibodies or low numbers of redblood or white blood cells. Samples of tissue from affected skin and kidneysshow characteristics of the disease.

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 at the same time) are extremely likely tohave SLE. The criteria are:

  • Butterfly rash
  • Discoid rash
  • Photosensitivity
  • Mouth ulcers
  • Arthritis
  • Inflammation of the lining of the lungs or the lining around the heart
  • Kidney damage
  • Seizures or psychosis
  • Certain types of anemia and low counts of certain white blood cells
  • Certain immune cells, anti-DNA antibodies, or a false-positive test for syphilis
  • Antinuclear antibodies

Treatment depends on the organ systems affected by SLE and the severity of the disease. Some patients have a mild form of SLE which responds to nonsteroidal anti-inflammatory drugs like ibuprofen (Motrin, Advil) and aspirin. Severeskin rashes and joint problems may respond to a group of medications usuallyused to treat malaria. More severely ill patients with potentially life-threatening complications (including kidney disease, heart inflammation, or nervous system complications), need to be treated with stronger drugs such as steroids. Because steroids have serious side effects, they are reserved for moresevere cases of SLE. Drugs that decrease the activity of the immune system also may be used for severely ill SLE patients.

Other treatments for SLE try to ease specific symptoms. Clotting disorders require blood thinners. Psychotic disorders require specific medications. Kidney failure may require either kidney dialysis (waste materials removed from the blood) or a kidney transplant.

A number of alternative treatments may help reduce the symptoms of SLE. Theseinclude acupuncture and massage for relieving the pain of sore joints and muscles. Stress management is important for people with SLE, including such techniques as meditation, hypnosis, and yoga. Dietary suggestions include 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 occur as a result of the digestive problems. Wheat, dairy products, and soy are themajor offenders. A diet rich in fish containing omega-3 fatty acids such as mackerel, sardines, and salmon may help. Because alfalfa sprouts have been associated with the onset of flares in SLE, they should be avoided.

Supplements may improve the health of SLE patients including vitamins B, C, and E, as well as selenium, zinc, magnesium, and a complete trace mineral supplement. Vitamin A is believed to help improve the coin-shaped skin rashes.

The prognosis for patients with SLE varies, depending on which organs are affected and how severe the inflammation is. Some patients have long periods oftime with mild or no symptoms. Between 90% and 95% of patients are still living after 2 years and about 82-90% of patients are still living after 5 years.After 10 years, between 71% and 80% of patients are still alive; between 63%and 75% are still alive after 20 years.

The most likely causes of death during the first 10 years include infectionsand kidney failure. After that, 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. About 30% of pregnant women with SLE will miscarry, and about 25% of all babies born to mothers with SLE are premature. While most babies born to mothers with SLE are normal, a rare condition called neonatal lupus causes a baby of an affected mother to develop a skin rash, liver or blood problems, and a serious heart condition.

There are no known ways to avoid getting SLE, but it's poissible to prevent the flareups by staying out of the sun, getting plenty of rest, 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-up (fever, increased fatigue, rash, headache) so that the person can try to prevent it from worsening.

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