Systemic Lupus Erythematosus

views updated May 21 2018

Systemic lupus erythematosus

Definition

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.

Description

The body's immune system is a network of cells and tissues responsible for clearing the body of invading organisms, like bacteria, viruses, and fungi. Antibodies are special immune cells that recognize these invaders, and begin a chain of events to destroy them. In an autoimmune disorder like SLE, a person's antibodies begin to identify 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, which are directed against the cell structure that contains genetic material (the nucleus), and anti-DNA antibodies, which are directed against genetic material (DNA).

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, such medications as hydralazine and procainamide can cause symptoms very similar to SLE. This condition is called drug-induced lupus. Drug-induced lupus usually disappears after the patient stops taking the particular medication.

Causes & 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. Susceptibility to SLE is known to have a genetic basis, although more than one gene is believed to be involved in disease development. As of 2002, notable progress has been made in narrowing the location of these genes. Because SLE patients may suddenly have worse symptoms (called a flare) after exposure to sunlight, such foods as alfalfa sprouts, and certain medications, environmental factors may also be at work.

The severity of symptoms varies over time, with periods of 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. Recurrent infections , particularly those caused by bacteria, are common in patients with SLE. 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 heartbeat (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. The molecular mechanism responsible for brain dysfunction in lupus was identified in 2001.
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

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 other 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.). A blood test called the lupus erythematosus cell preparation (or LE prep) test is also performed. The LE prep is positive in 70–80% of all patients with SLE. 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.

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 11 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

Although there is no cure for SLE, a number of alternative treatments may help reduce symptoms.

Acupuncture can relieve pain in joints and muscles.
Chinese herbals are chosen based on treatment principles and the patients specific symptoms. A simple decoction for the treatment of SLE joint and kidney problems is Lei Gong Teng (Caulis tripterygii ), Ji Xue Teng (Caulis spatholobi ), and Gan Cao (Radix glycyrrhizae ). Chinese patent medicines for SLE include Qin Jiao Wan (Gentiana Macrophylla Pill) and Kun Ming Shan Hai Tang Pian (Tripterygii Tablet).
DHEA (dehydroepiandrosterone) treatment, in a small study, led to disease improvement and reduction in the use of corticosteroids.
Diet. The SLE patient should drink plenty of water and eat a well balanced diet of whole, unprocessed foods that are low in fat and high in fiber. Mackerel, sardines, and salmon contain the beneficial fatty acid omega-3. Caffeine , sugar, alcohol, red meats, and alfalfa sprouts should be avoided. Because food allergies can be associated with SLE, an elimination/change in diet can help identify the offending foods (often wheat, dairy products, and/or soy).
Enzyme therapy treats SLE with 10X U.S.P. of digestive enzymes , protease, lipase, amylase, and cellulase to improve digestion of foods, based on the theory that a leaky gut causes SLE.
Exercise can reduce fatigue, reduce muscle weakness, speed weight loss, and increase energy, stamina, and confidence.
Herbals remedies include capsaicin (Capsicum species) cream, pau d'arco (Tabebuia species), pine (Pinus species) extract, wheat grass (Triticum aestivum ), Bupleurum falcatum, licorice (Glycyrrhiza glabra ), wild Mexican yam (Dioscorea villosa ), stinging nettle (Urtica dioica ), flaxseed (Linus usitatissimum ) oil, turmeric (Curcuma species), and borage (Borago officinalis ) oil.
Massage can relieve pain and reduce stress .
Probiotic treatment using Lactobacillus species to restore a healthy balance of bacteria in the intestines.
Stress management techniques, such as guided imagery, meditation, hypnotherapy , and yoga , can reduce stress that exacerbates SLE.
Supplements commonly recommended for SLE patients include vitamins B, C, and E, beta-carotene, bioflavonoids, selenium, zinc, magnesium , a complete trace mineral supplement, glutamine , gammaoryzanol, 1-butyrate, fructooligosaccharides (FOS), and omega-3 fatty acids (fish oil ). Vitamin A is believed to help improve discoid skin rashes.
Support groups for SLE patients can provide emotional and social help.

Allopathic treatment

Treatment depends on the organ systems affected and the severity of the disease. Patients with a mild form of SLE can be treated with nonsteroidal anti-inflammatory drugs, or NSAIDs, like ibuprofen (Motrin, Advil) and aspirin. 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 and possibly other drugs that decrease the activity of the immune system (immunosuppressant drugs).

Kidney failure may require the blood to be filtered by a machine (dialysis) or even a kidney transplantation.

Expected results

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 remission with mild or no symptoms. About 90–95% of patients are still living after two years with the disease, 82–90% after five years, 71–80% after 10 years, and 63–75% 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 development of abnormal blood clots is the most common cause of death.

For pregnant SLE patients, about 30% of the pregnancies end in miscarriage and about 25% of all babies are born prematurely. Most babies born to mothers with SLE are normal. Rarely, babies develop a condition called neonatal lupus, which is characterized by 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 to prevent flares include decreasing sun exposure, getting sufficient sleep, eating a healthy diet, decreasing stress, and exercising regularly.

Resources

BOOKS

Aaseng, Nathan. Autoimmune Diseases. New York: F. Watts, 1995.

Hahn, Bevra Hannahs. "Systemic Lupus Erythematosus." In Harrison's Principles of Internal Medicine. 15th ed., edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 2001.

Long, James W. The Essential Guide to Chronic Illness. New York: HarperPerennial, 1997.

Ravel, Richard. "Systemic Lupus Erythematosus (SLE)." In Clinical Laboratory Medicine: Clinical Application of Laboratory Data. St. Louis: Mosby, 1995.

Wallace, Daniel J. The Lupus Book. New York: Oxford University Press, 1995.

Ying, Zhou Zhong, and Jin Hui De. "Lupus Erythematosus." In Clinical Manual of Chinese Herbal Medicine and Acupuncture. New York: Churchill Livingston, 1997.

PERIODICALS

Graham, R. R., W. A. Ortmann, P. M. Gaffney, et al. "Localization of the Human SLE Susceptibility Genes Within the HLA Using a Recombinant Ancestral Haplotype Approach. American Journal of Human Genetics 69 (October 2001): 507.

"Lupus Brain Damage Pathway Identified Through Molecular Mechanism." Immunotherapy Weekly (December 12, 2001): 7.

Mann, Judy. "The Harsh Realities of Lupus." The Washington Post 120 (October 8, 1997): C12.

Noel, V., O. Lortholary, P. Casassus, et al. "Risk Factors and Prognostic Influence of Infection in a Single Cohort of 87 Adults with Systemic Lupus Erythematosus." Annals of the Rheumatic Diseases 60 (December 2001): 1141–1144.

Umansky, Diane. "Living with Lupus." American Health for Women 16 (June 1997): 92+.

Yap, Hui-Kim, Siau-Gek Ang, Yee-Hing Lai, Vinod Ramgolam, and Stanley C. Jordan. "Improvement in Lupus Nephritis Following Treatment with a Chinese Herbal Preparation." Archives of Pediatric and Adolescent Medicine 153 (August 1999): 850–852.

ORGANIZATIONS

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

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

OTHER

Balch, T. Stephen. "Living Well with Lupus." January 1, 2001 [cited October 2002]. <http://www.hamline.edu/~lupus/articles/living_well_with_lupus.html>.

Hoffman, David L. "The Use of Herbs in the Treatment of Systemic Lupus Erythematosus." HealthWorld Online. [cited October 2002]. <http://www.healthy.net>.

Belinda Rowland

Rebecca J. Frey, PhD

Gale Encyclopedia of Alternative Medicine Rowland, Belinda; Frey, Rebecca

Systemic Lupus Erythematosus

views updated May 21 2018

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.

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.

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).
When 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

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

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).