Systemic Lupus Erythematosus

Systemic lupus erythematosus is a chronic inflammatory autoimmune disorder which may affect many organ systems including the skin, joints and internal organs. The disease may be mild or severe and life-threatening. African-Americans and Asians are disproportionately affected.

Information about Systemic Lupus Erythematosus

Normally the immune system controls the body's defenses against infection. In systemic lupus erythematosus (SLE) and other autoimmune diseases, these defenses are turned against the body and rogue immune cells attack tissues. Antibodies may be produced that can react against the body's blood cells, organs, and tissues. These lead immune cells to attack the affected systems, producing chronic disease.

The mechanism or cause of autoimmune diseases is not fully known, but many researchers suspect it occurs following infection with an organism that looks similar to particular proteins in the body, which are later mistaken for the organism and wrongly targeted for attack.

The disease affects 9 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 years. SLE may also be caused by certain drugs. When this occurs, it is known as drug-induced lupus erythematosus and it is usually reversible when the medication is stopped.

The course of the disease may vary from a mild episodic illness to a severe fatal disease. Symptoms also vary widely in a particular individual over time and are characterized by periods of remission and exacerbation. At its onset, only one organ system may be involved. Additional organs may become involved later. The following organ system manifestations may be seen, but other manifestations are possible.

Musculoskeletal
Almost all people with SLE have joint pain and most develop arthritis. Frequently affected joints are the fingers, hands, wrists, and knees. Death of bone tissue can occur in the hips and shoulders and is frequently a cause of pain in those areas.

Skin
A "butterfly" rash over the cheeks and bridge of the nose affects about half of those with SLE. The rash is usually worsened by sunlight. A more diffuse rash may appear on other body parts that are exposed to the sun. Other skin lesions or nodules can also occur.

Kidney
Some people with SLE have deposits of protein within the cells (glomeruli) of the kidney. However, less than 50% have lupus nephritis as defined by persistent inflammation (irritation and swelling) in the kidney. These patients may eventually develop renal failure and require dialysis or kidney transplantation.

Nervous system
Neurologic disorders can affect up to 25% of those with SLE. Mild mental dysfunction is the most common symptom, but any area of the brain, spinal cord, or nervous system can be affected. Seizures, psychosis, organic brain syndrome, and headaches are some of the varied nervous system disorders that can occur.

Blood
Blood disorders can affect up to 85% of those with SLE. Venous or arterial blood clots can form and are associated with strokes and pulmonary embolism, or pregnancy loss. Predisposition to blood clots, or less commonly bleeding, occurs due to antibodies against lipids involved in blood clotting and is referred to as anti-phospholipid antibody syndrome (APS). (See Lupus anticoagulant.) Often platelets are decreased which may cause bleeding or clotting problems. Anemia of chronic disease often develops at some point in the course SLE.

Heart
Inflammation of various parts of the heart may occur as pericarditis, endocarditis, or myocarditis. Chest pain and arrhythmias may result from these conditions.

Lungs
Pleurisy, an inflammation of the lining of the lung, and pleural effusions, a fluid collection between the lung and its lining, can occur as a result of SLE. Chest pain and shortness of breath are frequently results of these disorders.

African Americans and Asians are affected more often than people from other races.

Symptoms of  Systemic Lupus Erythematosus

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.

• Joints. Joint pain and problems, including arthritis, are very common. About 90% of all SLE patients have these types of problems.
• Skin. A number of skin rashes may occur, including a red butterfly-shaped rash that spreads across the face. The "wings" of the butterfly appear across the cheekbones, and the "body" appears across the bridge of the nose. A discoid, or coin-shaped, rash causes red, scaly bumps on the cheeks, nose, scalp, ears, chest, back, and the tops of the arms and legs. The roof of the mouth may develop sore, irritated pits (ulcers). Hair loss is common. SLE patients tend to be very easily sunburned (photosensitive).
• Lungs. Inflammation of the tissues that cover the lungs and line the chest cavity causes pleuritis, with fluid accumulating in the lungs. The patient frequently experiences coughing and shortness of breath.
• Heart and circulatory system. Inflammation of the tissue surrounding the heart causes pericarditis; inflammation of the heart itself causes myocarditis. These heart problems may result in abnormal beats (arrhythmias), difficulty pumping the blood strongly enough (heart failure), or even sudden death. Blood clots often form in the blood vessels and may lead to complications.
• Nervous system. Headaches, seizures, changes in personality, and confused thinking (psychosis) may occur.
• Kidneys. The kidneys may suffer significant destruction, with serious life-threatening effects. They may become unable to adequately filter the blood, leading 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 one of the nerves responsible for vision may cause vision problems, and blindness can result from inflammation of the blood vessels (vasculitis) that serve the retina.

Diagnosis of  Systemic Lupus Erythematosus

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:

• Butterfly rash
• Discoid rash
• Photosensitivity
• Mouth ulcers
• Arthritis
• Inflammation of the lining of the lungs or the lining around the heart
• Kidney damage, as noted by the presence of protein or other abnormal substances called casts in the urine
• Seizures or psychosis
• The presence of certain types of anemia and low counts of particular white blood cells
• The presence of certain immune cells, anti-DNA antibodies, or a falsely positive test for syphilis
• The presence of antinuclear antibodies.

Treatment of  Systemic Lupus Erythematosus

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 of  Systemic Lupus Erythematosus

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 the 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 of  Systemic Lupus Erythematosus

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 of  Systemic Lupus Erythematosus

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.