Lupus, also known as lupus erythematosus, is an autoimmune inflammatory disorder that occurs mostly in women.
Lupus produces widely varying symptoms, although joint pain is reported by most patients and skin lesions are common. Lupus can cause short periods of symptoms alternating with healthy periods, or can progress into a life-threatening disorder affecting the heart, kidneys, and other organs.
Why the disease is termed lupus is unknown, but it has been known as a distinct disorder and called lupus by European physicians since at least the tenth century a.d. The term erythematosus was first attached to the disease in the 1850s, and it refers to the patchy congestion of skin capillaries with blood (erythema) that often accompanies the disease.
Between one million and 1.5 million Americans have some form of lupus. The incidence among women is 10–15 times greater than among men, and it is two to three times more common among African Americans, Hispanics, Asians, and Native Americans than among whites. Lupus most often appears for the first time in women between the ages of 15 and 44. Twenty thousand people die of lupus-related causes in the United States annually.
Causes and symptoms
Lupus is an autoimmune disorder, a disease in which the body's immune system turns against the body itself. In a healthy person, the immune system defends against invading organisms but does not, in general, attack the body's own tissues. The cause of lupus is unknown. However, it is known that lupus has a genetic component, which means a predisposition to lupus can be inherited. Approximately 10% of lupus patients have one or more direct relatives with lupus. (Note that this means that 90% of lupus patients have no such relatives; however, it shows a
genetic connection because 10% is a much higher figure for familial lupus than can be attributed to chance alone.) Lupus has been definitely linked to genes on chromosome 1 and less certainly to genes on chromosomes 4 and 6.
Given genetic susceptibility, the disease may either develop spontaneously or be triggered by some environmental factor. Environmental factors known to trigger lupus include infections (e.g., Epstein-Barr virus, which infects 99% of children with lupus, but only 70% of healthy children), antibiotics, ultraviolet light (the rays in sunlight or sunlamp-light that causes sunburn), stress, smoking, certain medications, and hormones (especially estrogen, the female sex hormone).
Lupus manifests as a continuum or spectrum of disorders. However, it is common to divide lupus cases into four categories or groups:
- Systemic lupus erythematosus. This is the most serious form of lupus and affects about 70% of all persons with lupus. It is termed systemic because, in this variety of lupus, the body's immune system attacks one or more essential body systems. Targets may include the brain, kidneys, heart, pancreas, or other organs.
- Discoid or cutaneous lupus erythematosus. This variety of lupus is less severe, in that it attacks the skin only. However, it can be disfiguring, often attacking the skin of the face. The term discoid is derived from the round (disc-shaped) lesions that appear on the skin. About 10–15% of lupus patients have cutaneous lupus.
- Drug-induced or drug-related lupus erythematosus. This term refers to lupus that develops after a patient has taken a medication. Medications that can trigger drug-induced lupus include procainamide or hydralazine. Many of the substances that can potentially trigger lupus fall into the class of aromatic amines, or hydrazines. For example, the aromatic amine paraphenylenediamine is present in certain hair dyes and has been associated with lupus or lupus-like syndrome. Tartrazine (a food coloring, FD&C yellow No. 5), which is present in thousands of foods and medications, has also been associated with lupus. Cocaine abuse can induce lupus and several other connective-tissue diseases, as can exposure to certain metals (e.g., mercury). Between 10,000 and 15,000 people are diagnosed with drug-induced lupus annually in the United States.
- Mixed connective tissue disease. Approximately 10% of patients with lupus also have symptoms of one or more additional connective-tissue diseases.
The symptoms of lupus are quite varied. In discoid lupus, red patches (erythema) appear symmetrically on the cheeks, possibly extending to the face, neck, scalp, and other parts of the body. No organ other than the skin is affected (or the disease is classified as systemic, rather than discoid). Systemic lupus may begin suddenly, signaled by fever, or develop slowly over months or years. Chronic fatigue is a common symptom. Symptoms related to impairment of any organ may occur. The lupus disease process in a given organ is named after that organ; for example, inflammation of the kidneys is termed lupus nephritis, and inflammation of the brain is termed lupus cerebritis. Kidney involvement may be fatal. Over 50% of all systemic lupus patients in the United States presently have some degree of lupus cerebritis; 25–75% have neuropsychiatric symptoms at some time in their illness. Symptoms of lupus cerebritis may include headaches, seizures , stroke , psychosis, dementia , peripheral neuropathy , cerebellar ataxia (failure of muscular coordination, usually on one side of the body), chorea (jerky, involuntary movements), and others. Duration of central nervous system involvement may be transient (as with a migraine headache ) or long lasting (as with dementia). Stroke incidence is 3–20% in systemic lupus patients, and is highest in the first five years of the disease. Peripheral neuropathy (carpal tunnel syndrome , for example) occurs in more than 20% of systemic lupus patients and cranial nerve palsies occur in 10–15%.
Exposure to the ultraviolet rays in sunlight can trigger lupus or, in a person who already has the disease, cause it to flare up. Worsening flare-ups of the disease can be life threatening because they can include inflammation and failure of the kidneys. Also, declining memory and mental sharpness with long-term lupus is common.
Lupus is notoriously difficult to diagnose. Many cases are not diagnosed until the patient has suffered irreversible kidney damage; for patients who do not have organ-threatening disease, diagnosis takes an average of two years of searching among physicians and conditions. The telltale erythematous skin lumps or rashes that give lupus erythematosus the latter half of its name eventually appear in 90% of systemic lupus patients and all discoid lupus patients, but may not appear early enough in the course of the disease to guarantee timely diagnosis. Additionally, no single lab test can confirm lupus, although certain antibody tests can help to distinguish lupus from other diseases.
Diagnosis of systemic lupus is based on a list of 11 criteria listed by the American College of Rheumatology. If four or more of the 11 criteria are met, a patient is deemed to have systemic lupus. The criteria include discoid or macular rash (often in a classic facial butterfly pattern across the nose and cheeks), photosensitivity, ulcers in the mouth, kidney dysfunction, and the presence of various blood factors such as anti-DNA antibody or anti-nuclear antibody (antibody that targets cell nuclei).
Approximately 15% of diagnoses of lupus may be misdiagnoses of other disorders, including fibromyalgia, seronegative spondyloarthropathies such as ankylosing spondylitis or Reiter's syndrome, autoimmune thyroiditis, and multiple sclerosis .
Although diagnosis of lupus cerebritis is particularly difficult, even if a patient has lupus, this does not necessarily mean that the neurological symptoms are due to lupus. Imaging studies cannot necessarily distinguish lupus cerebritis, although magnetic resonance imaging (MRI) studies are considered helpful. Positron emission tomography (PET) imaging has a high sensitivity to changes in the brain resulting from lupus cerebritis.
As with other neurological diseases in which the spectrum of symptoms varies widely, the treatment team must be designed for each individual case of lupus. A dermatologist will be involved if skin lesions are present; a neurologist , if cognitive loss is a possibility; a nephrologist will monitor kidney function; and a rheumatologist is often involved because of the frequency of joint pain. Other specialists will be needed depending on what organ systems are affected.
There is no known cure for lupus. However, there are numerous interventions designed to lessen the severity of the disease. These interventions can be classed as pharmacologic (drug-based) or nonpharmacologic.
Pharmacologic interventions (drug therapies)
Five categories of medication are used to treat systemic lupus patients: sunscreens and steroid lotions, nonsteroidal anti-inflammatory drugs (NSAIDs, e.g., acetaminophen or ibuprofen), corticosteroids (e.g., prednisone to suppress the autoimmune response and control inflammation), anti-malarial drugs, and cytotoxic agents (i.e., chemotherapy drugs that are used for cancer, such as methotrexate, azathioprine, and cyclophosphamide).
Cytotoxic agents are used in order to decrease steroid dosage. Anticoagulants (blood thinners) may also be prescribed. For patients with non-organ-threatening disease, the antimalarial drug hydroxychloroquine is often prescribed; prednisone is often prescribed in cases of organ-threatening disease. New lupus drugs are under investigation; with recent increases in knowledge about the genetic and molecular basis of autoimmune disorders, including lupus, pharmacological treatment breakthroughs are possible at any time.
Nonpharmacologic (non-drug) interventions
All persons with lupus should guard against exposure to the sun and use protective clothing, sunscreen, and common sense when going outdoors. Adequate exercise can protect against fatigue, obesity, osteoporosis (weakening of the bones), and hyperlipidemia (excessive fats in the blood plasma). In some cases, dietary restrictions may be helpful, including especially the avoidance of food allergens and foods that may trigger lupus symptoms (such as alfalfa seeds). Vitamins, minerals, and dietary fatty acids have been shown to moderate lupus symptoms in some cases. On the other hand, some dietary supplements such as melatonin and Echinacea can worsen symptoms of some autoimmune diseases.
For lupus cerebritis, therapy choices include all the above options for alleviating the disorder throughout the rest of the body. Drug therapy can also include psychotropic medications such as antipsychotics, antidepressants, and benzodiazepines to stabilize mood, if this is affected. Unfortunately, long-term use of corticosteroids, one of the mainstays of pharmacological lupus treatment, may itself cause psychiatric symptoms. Experimental investigation of pheresis of cerebrospinal fluid for treatment of lupus cerebritis (cerebrospinal fluid is withdrawn from, filtered, and returned to the patient) was begun in the early 1990s.
As of mid-2004, approximately 25 lupus-related clinical trials were in progress, including investigations of monoclonal antibody therapy, the genetics of lupus, quality-of-life improvement, ultraviolet light therapy, stem-cell transplantation therapy, the mechanisms of kidney and brain damage, and many other aspects of lupus. Updated information on these trials can be found at the National Institutes of Health clinical trials website at <http://www.clinicaltrials.gov> for up-to-date information.
Prognosis for the individual patient depends on the severity of the disease process. Lupus can be fully compatible with a normal lifespan, or can result in fatal organ failure, depending upon the progression of the disorder in each individual.
Before corticosteroids became available, half of all patients with systemic lupus died within two years. Today, half of systemic lupus patients with organ-threatening complications survive for 20 years or longer. However, most systemic lupus patients eventually die from infections or from heart disease complicated by long-term use of corticosteroids.
There is some evidence that lupus may spontaneously resolve in part or whole, or resolve in response to treatment, in some lupus patients who have had the disease long term (i.e., 10 years or more).
Psychological counseling may be helpful, given that a diagnosis of lupus is life altering, and stress and frustration can enhance symptoms while searching for a diagnosis. Genetic counseling may be appropriate, as children of women with lupus have a 10% chance of developing lupus if female and 2% if male, while 20% of offspring overall will develop an autoimmune disorder of some type.
Phillips, Robert H., et al. Coping with Lupus: A Practical Guide to Alleviating the Challenges of Systemic Lupus Erythematosus, 3rd ed. New York: Avery Penguin Putnam, 2001.
Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. New York: Oxford Press, 2000.
Marshall, Eliot. "Lupus: Mysterious Disease Holds Its Secrets Tight." Science (April 26, 2002).
Nickens, Candice. "Treating Systemic Lupus Erythmatosus." Minority Health Today (July 1, 2000).
Rushing, Jill D. "Managing Organ-threatening Systemic Lupus Erythematosus." MedSurg Nursing (December 1, 2003).
"Systemic Lupus Erythematosus: Guidelines for Control." Consultant (February 1, 2000).
"NINDS Neurological Sequelae Of Lupus Information Page." National Institute of Neurological Disorders and Stroke. April 24, 2004 (June 1, 2004). <http://www.ninds.nih.gov/health_and_medical/disorders/lupus_doc.htm>.
Lupus Foundation of America. 2000 L Street, N.W., Suite 710, Washington, DC 20036. (202) 349-1155; Fax: (202) 349-1156. <http://www.lupus.org/>.
"Lupus." Gale Encyclopedia of Neurological Disorders. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/science/encyclopedias-almanacs-transcripts-and-maps/lupus
"Lupus." Gale Encyclopedia of Neurological Disorders. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/science/encyclopedias-almanacs-transcripts-and-maps/lupus
lupus (lōō´pəs), noninfectious chronic disease in which antibodies in an individual's immune system attack the body's own substances. In lupus, known medically as lupus erythematosus, antibodies are produced against the individual's own cells, causing tissue inflammation and cell damage. Because the vascular and connective tissue of any body organ may be affected, various symptoms may result. Generalized symptoms include fever, weakness, weight loss, anemia, enlargement of the spleen, and a characteristic butterfly-shaped skin rash on the face. Heart, joint, and kidney disease are common (see nephritis). It is believed that the disease may be triggered by certain drugs or foreign proteins, exposure to ultraviolet radiation, or extreme stress. The disease, which may range from mild to fatal, occurs commonly in young women. It is treated with immunosuppressive drugs and steroids. See immunity; autoimmune disease.
See R. G. Lahita and R. H. Phillips, Lupus: Everything You Need to Know (1998).
"lupus." The Columbia Encyclopedia, 6th ed.. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/reference/encyclopedias-almanacs-transcripts-and-maps/lupus
"lupus." The Columbia Encyclopedia, 6th ed.. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/reference/encyclopedias-almanacs-transcripts-and-maps/lupus
www.uklupus.co.uk Details of SLE from The Lupus Site
"lupus." A Dictionary of Nursing. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/caregiving/dictionaries-thesauruses-pictures-and-press-releases/lupus
"lupus." A Dictionary of Nursing. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/caregiving/dictionaries-thesauruses-pictures-and-press-releases/lupus
Lupus is a disease in which a person's immune system attacks the body's own organs and tissues (see autoimmune disorders entry). The complete medical name for lupus is systemic lupus erythematosus (SLE; pronounced LOO-puhs er-uh-THEM-uh-tuhs).
The immune system is a network of cells and tissues that protect the body against foreign organisms, such as bacteria and viruses. One mechanism used by the immune system is the release of antibodies. Antibodies are molecules that attack and destroy foreign organisms. For each type of organism, the immune system produces a special kind of antibody.
In a patient who has lupus, the immune system functions incorrectly. It thinks that the body's own cells are foreign organisms and releases antibodies to attack these cells the way it would attack bacteria and viruses. This causes tissues to become inflamed (red and swollen). They may even be killed by the attacking antibodies.
Lupus occurs in both males and females of all ages, but it is much more common in women. About 90 percent of all lupus cases occur in women. The majority of these women are of childbearing age. African Americans are more likely to develop lupus than Caucasians (whites).
The cause of lupus is unknown. Some researchers think that heredity may be a factor. Environmental factors also may be involved. In some cases, the symptoms of lupus become worse after exposure to sunlight, alfalfa sprouts, and certain medications. On rare occasions, a form of lupus can be caused by medications. Some drugs used to treat heart problems are among these medications. The lupus usually disappears when the person stops taking the drug.
Lupus: Words to Know
- Autoimmune disorder:
- A condition in which a person's immune system mistakes the body's own tissues for foreign invaders and begins to make antibodies against them.
- Immune system:
- A network of cells and tissues that work together to protect the body against foreign invaders, such as bacteria and viruses.
- Extremely disordered thinking accompanied by a poor sense of reality.
The symptoms of lupus vary in seriousness. Sometimes they are quite mild, and sometimes they are quite severe. Typical symptoms include fever, fatigue, muscle pain, decreased appetite, and weight loss. The spleen and lymph nodes are often swollen. Other areas that may be affected by lupus include:
- Joints. Joint pain and disorders, such as arthritis (see arthritis entry), are common. About 90 percent of all lupus patients have such problems.
- Skin. Lupus may cause skin rashes on any part of the body. They usually occur on the face, scalp, chest, ears, back, arms, and legs. When they occur in the mouth, they form ulcers (open sores). Hair loss is common.
- Lungs. Lupus may cause inflammation of the pleura, the tissue that lines the lungs. The patient may experience coughing and shortness of breath.
- Heart and circulatory system. Lupus may cause inflammation of the tissue surrounding the heart (pericarditis; pronounced per-i-kar-DIE-tiss) or of the heart itself (myocarditis; pronounced my-o-kar-DIE-tiss). When this happens, various heart problems may develop, such as an irregular heartbeat (arrhythmia; pronounced uh-RITH-mee-uh), heart failure, and even sudden death. Blood clots often form in the blood vessels. These blood clots can break loose and cause a stroke (see stroke entry) or other complications.
- Nervous system. Headaches, seizures, personality changes, and psychosis (confused thinking) may occur.
- Kidneys. During a lupus attack, the body's kidney cells may begin to die. When this happens, the kidney can no longer filter blood. Toxins (poisons) may build up in the kidney, causing it to stop functioning.
- Gastrointestinal (digestive) system. Patients may experience nausea, vomiting, diarrhea, and abdominal (stomach) pain. The lining of the stomach may also become inflamed.
- Eyes. The eyes may become red, sore, and dry. Inflammation of nerves in the eye may cause vision problems and blindness.
Lupus is usually difficult to diagnose, especially since many of its symptoms are similar to other diseases, such as rheumatoid arthritis (see arthritis entry) and multiple sclerosis (see multiple sclerosis entry). There is no one test that can be used to diagnose the disease. Blood tests can be used to look for certain kinds of antibodies. The most dependable of these tests may be correct 70 percent to 80 percent of the time.
Many doctors rely on a standard created by the American Rheumatism Association to diagnose lupus. According to this standard, a patient has to have four of eleven symptoms to be diagnosed with lupus. Those symptoms are:
- Butterfly rash, a distinctive type of facial rash
- Discoid rash, another distinctive type of facial rash
- Unusual sensitivity to light
- Ulcers in the mouth
- Inflammation of the lining of the lungs or the lining around the heart
- Kidney damage
- Seizures or psychosis
- Low numbers of red blood cells or certain types of white blood cells
- The presence of certain kinds of immune cells
- The presence of certain kinds of antibodies
Treatment of lupus depends on how serious a patient's case is. Mild cases may involve rashes and moderate pain. These cases can be treated with nonsteroidal anti-inflammatory drugs, such as aspirin and ibuprofen (pronounced i-byoo-PRO-fuhn, trade names Motrin, Advil). More serious rashes and joint problems may be treated with drugs also used to treat malaria (see malaria entry).
More serious symptoms may have to be treated with steroids. Steroids can reduce inflammation and swelling. They have some serious side effects, however, so they must be used with caution.
The most seriously ill patients may be treated with immunosuppressant drugs. Immunosuppressant drugs cause the immune system to shut down partially or completely. These drugs also have very serious side effects. With a weakened immune system, a patient is at risk for many other kinds of infections.
Other lupus treatments are designed for specific systems affected by the disease. For example, substances that thin the blood can be used if blood clots have formed. A person whose kidneys have begun to fail may require kidney dialysis or even a kidney transplantation. Kidney dialysis is a process in which a machine artificially cleanses a person's blood.
A number of alternative treatments have been suggested to help reduce the symptoms of lupus. These include acupuncture and massage for relieving the pain of sore joints and muscles. Patients can be taught to relax with techniques such as meditation and yoga. Hydrotherapy (water therapy) may also promote relaxation.
Proper nutrition may be an important factor in treating the symptoms of lupus. Some cases of the disease may be triggered by certain types of foods. Wheat, dairy products, and soy are the most common causes of the disease. Avoiding these foods may reduce the symptoms of lupus.
Some practitioners recommend nutritional supplements, such as vitamins B, C, and E, and minerals, such as magnesium, selenium, and zinc. They think these supplements may improve the general health of patients with lupus. Vitamin A can also be used to treat facial rashes.
Herbalists believe that certain herbs help relieve specific symptoms of lupus. They think that herbs can also help people develop a healthier outlook on life.
The prognosis for lupus depends primarily on two factors: the systems affected and the degree of inflammation. Some patients experience mild symptoms or no symptoms at all over long periods of time. About 90 percent to 95 percent of all patients with lupus are still alive two years after diagnosis. Up to 75 percent of all patients survive twenty years or more.
The most common causes of death in the early years of lupus are infectious diseases and kidney failure. For people who have had the condition longer, the most common cause of death is blood clots.
There is no way to avoid developing lupus. However, patients with the condition can often avoid the worst symptoms of the disease by maintaining a healthy diet, getting plenty of rest, avoiding stress, exercising regularly, and decreasing exposure to the sun. Patients can try to find out what factors seem to worsen their symptoms and then avoid those factors as much as possible.
FOR MORE INFORMATION
Aaseng, Nathan. Autoimmune Diseases. New York: Franklin Watts, 1995.
Lahita, Robert G., and Robert H. Phillips. Lupus: Everything You Need to Know. Garden City Park, NY: Avery Publishing Group, 1998.
Wallace, Daniel J. The Lupus Book. New York: Oxford University Press, 1995.
Mann, Judy. "The Harsh Realities of Lupus." Washington Post (October 8, 1997): p. C12.
Umansky, Diane. "Living with Lupus." American Health for Women (June 1997): p. 92+.
American College of Rheumatology. 1800 Century Place, Suite 250, Atlanta, GA 30345. (404) 633-3777. http://www.rheumatology.org
Lupus Foundation of America, Inc. 1300 Piccard Drive, Suite 200, Rockville, MD 20850–4303. (800) 558-0121. http://www.lupus.org.
Lupus Network. 230 Ranch Drive, Bridgeport, CT 06606. (203) 372-5795.
"Lupus." UXL Complete Health Resource. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/news-wires-white-papers-and-books/lupus
"Lupus." UXL Complete Health Resource. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/news-wires-white-papers-and-books/lupus
Lupus (LOO-pus) is a chronic (long-lasting) disease that causes inflammation of connective tissue, the material that holds in place the various structures of the body.
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“Oh, not again!” shouted 18-year-old Julia, her toothbrush falling into the sink with a clatter. It was the fifth morning this week that it had slipped from her hands. She had been waking up with achy, puffy hands, and she was finding it hard to grip things with her stiff fingers. A few weeks later, when filling out a form for a back-to-school physical, she wrote about her stiff hands and also mentioned that she had been feeling really tired all summer. Her doctor suggested she get some blood tests and called Julia’s mother a few days later with a referral to a rheumatologist*. He suspected that Julia had lupus.
- * rheumatologist
- (roo-ma-TOL-o-jist) is a doctor who specializes in disorders involving the connective tissue structures of the body.
Lupus causes inflammation of connective tissues in the body. Connective tissue is the material that holds the various structures of the body in place. The cause or causes of lupus are uncertain. However, it is believed to be an autoimmune disorder, which is a reaction of the immune system against one’s own body. The medical name for lupus is lupus erythematosus (er-i-thee-ma-TO-sus).
There are two main types of lupus. These are discoid lupus erythematosus (DLE), which mostly causes reddened patches on the skin, and systemic lupus erythematosus (SLE), which affects the skin but also involves other tissues and organs.
A third condition, called lupus vulgaris, is unrelated to lupus erythematosus. It is a rare form of tuberculosis that typically produces nodules, or small lumps, on the skin.
Lupus is a disorder that occurs in people of all ages worldwide. In the United States, it has been estimated that there are half a million lupus patients, or about one person in every 600.
Although members of both sexes can get lupus, the great majority of people affected by the disease are women (at least 9 out of 10 in the case of SLE, and approximately 7 out of 10 in the case of DLE). The majority of women affected are young to middle-aged. Like Julia, about half of SLE patients show their first signs of the disorder between the ages of 15 and 25.
In the United States, lupus is about three times more prevalent in people of African ancestry than in people of European ancestry. It also appears to be more prevalent in people of American Indian and Asian origin.
Lupus is not a contagious disease. One person cannot catch it from another, and there is no need to avoid being near someone who has lupus.
In about 10 percent of cases, certain prescription drugs, such as those used for irregular heartbeat or high blood pressure, can cause symptoms of SLE. (The symptoms usually go away when the drugs are discontinued.) This effect is noted most often in elderly patients and is referred to as drug-induced lupus. In the other 90 percent of SLE cases and in all instances of DLE, the cause or causes of the disorder are not known with certainty.
Although most people who have studied lupus agree that it is an autoimmune disorder, this knowledge provides only a part of the explanation of its cause. The reason is that the causes of immune disorders are not themselves very well understood. There are, however, some things that doctors believe can play a role in the development of lupus.
Heredity is assumed to play a part, although it is not known exactly what its role is. It has been determined that a person with a close relative who has SLE is slightly more likely to develop the disease (by about 10 percent) than someone who does not. The likelihood increases if one has an identical twin with SLE.
Its higher prevalence in some racial groups also suggests that heredity plays a role. However, no gene (hereditary factor) has been specifically linked to the development of lupus.
The fact that women are much more likely to get lupus than men suggests that hormones also may be associated with the disorder.
Certain environmental influences are believed to play a part in the onset of lupus. It is believed that autoimmune responses in some individuals may be triggered by bacteria, viruses, extreme stress, sunlight, certain antibiotics, or food additives. But not everyone gets lupus because they caught a particular virus or bacteria, are stressed about something in their lives, took a certain antibiotic, or ate a certain food.
The antibodies involved in the autoimmune response are called “autoantibodies.” They are thought to react specifically with certain chemical constituents of the cells in the body, producing inflammation and damage to organs and tissues.
Did You Know?
- The name “lupus erythematosus” comes from lupus, which is Latin for “wolf,” and “erythema,” which refers to reddened skin. In the past, people thought that patients with the facial rash looked as though they had been bitten or scratched by a wolf.
- The large majority of lupus patients are women.
- Lupus is not contagious.
- There is no one symptom or test that means someone has lupus.
- Many lupus patients develop arthritis.
- Many people with lupus have to stay out of the sun.
- Most lupus patients can lead nearly normal lives.
The signs and symptoms of lupus vary greatly in different individuals, both in the parts of the body involved and the degree of severity. The symptoms may also come and go, and may go away for weeks or months. Although lupus occurs much more frequently in women than in men, the symptoms in males are no less severe than in females.
Discoid Lupus Erythematosus
The mildest and most common form of lupus, DLE usually involves only the skin. It produces a rash of thickened, scaly reddish patches on the face and sometimes other parts of the body. Often the rash spreads in a characteristic butterfly-shaped pattern over the cheeks and bridge of the nose. After a few weeks or months, when healing has taken place, dark-colored or pale scars may remain. If the condition extends to the scalp, the person may lose some of his or her hair.
Sunlight tends to trigger and worsen the rash of lupus. In DLE, the patches sometimes nearly disappear during the winter months. Most people who have DLE are otherwise in good health. The condition only rarely progresses to SLE.
Systemic Lupus Erythematosus
People with SLE may have the same type of rash as those with DLE, and they may lose hair as well. In SLE, however, the skin lesions may spread and cause damage to the mucous membranes and other tissues. In some SLE patients the skin is not affected.
Arthritis may be the first symptom in some people with SLE. In fact, SLE is medically classified in the same family of diseases as rheumatoid arthritis, a disorder that causes painful inflammation in the joints. Other early symptoms may include weakness, extreme fatigue, fever, sensitivity to sunlight, and loss of weight.
Internal organs also may be affected by SLE, often causing serious disorders. Problems with kidney function are common in SLE patients, and uremia (yoo-REE-me-a), or the buildup of toxic substances in the blood due to kidney failure, can be fatal. The nervous system may be affected, causing psychological problems, seizures, or other symptoms. The lungs, heart, liver, and blood cells may also be involved.
In the blood, the presence of certain antibodies called anti-phospholipid (AN-ti-fos-fo-LIP-id) antibodies interferes with the normal function of the blood vessels, and can bring on a stroke or heart attack. In pregnant women, the presence of these antibodies can cause a miscarriage.
Lupus can be hard to diagnose, especially if there are just a few symptoms. There is no one sign or symptom that definitely means someone has lupus, and there is no single laboratory test that will diagnose it either. For this reason, a combination of observations and tests is always necessary.
What Is an Autoimmune Disorder?
Nearly everyone is familiar with allergies such as hay fever and asthma, either in their own experience or that of friends or family. An autoimmune disorder might best be understood as a kind of allergy, except that the immune system attacks parts of one’s own body instead of outside substances such as dust and pollen.
The normal function of the immune system is to protect the body from invading microorganisms or toxic substances. In order to perform this function, it produces antibodies and special white blood cells (lymphocytes) that will recognize and destroy the intruders.
In autoimmune disorders, these responses (for reasons that are as yet not fully understood) occur against the body’s own cells, tissues, and organs. This reaction can produce a number of illnesses, including rheumatoid arthritis, a type of diabetes, and lupus.
Diagnostic tests for SLE include blood tests for certain antibodies that attack the nucleus of cells, and for LE cells. LE (lupus erythematosus) cells are white blood cells that destroy other blood cells and are an indication of lupus. Sometimes a skin biopsy (removal of a small sample) is performed to examine for antibodies active in lupus.
Early diagnosis is important so that treatment can be started as soon as possible.
Various types of treatment may be chosen depending upon the particular needs and symptoms of lupus patients. A variety of drugs are prescribed to counteract pain, inflammation, and related problems.
NSAIDs (nonsteroidal anti-inflammatory drugs), such as aspirin, ibuprofen, and naproxen, are used to lessen pain and inflammation in the joints and muscles. Another group of medications, called corticosteroids (kor-ti-ko-STEER-oids), is prescribed to reduce inflammation and activity of the immune system. A third group, called anti-malarials because they also are used to treat malaria, is often prescribed for skin and joint symptoms. Skin eruptions are treated with ointments and creams that contain corticosteroids and sunscreens.
Drugs prescribed to treat lupus can often cause unwanted side effects. For this reason, and because symptoms may change, ongoing medical advice with regular checkups is needed.
There is no cure for lupus, but effective treatment can maintain normal body function and control symptoms in the great majority of patients. Nonetheless, SLE can be a life-threatening disease, particularly when the kidneys are involved. The most common causes of death are kidney failure, bacterial infection, and heart failure.
There is no specific preventive measure, like vaccination, that one can take to keep from getting lupus. If one is diagnosed with lupus, however, there are ways to reduce the likelihood of “flares,” or sudden worsening of symptoms. Patients who are sensitive to sunlight can help prevent rashes by avoiding excessive exposure to the sun, using sunscreens, or wearing broad-brimmed hats. Although there is no vaccination for lupus itself, immunization against other infections is recommended.
Lifestyle changes can help patients with lupus avoid or control symptoms as well. Regular exercise can prevent some muscle weakness and fatigue. Smoking and excessive alcohol intake can be harmful, as they are to anyone. Changing such negative habits can bring about general improvement in one’s health. Joining support groups and talking with family, friends, and physicians can ease the effects of stress.
Lahita, Robert G., and Robert H. Phillips. Lupus: Everything You Need to Know. Garden City, NY: Avery Publishing Group, 1998. A nontechnical guide written in a question-and-answer format.
Blau, Sheldon Paul, and Dodi Schultz. Living with Lupus: All the Knowledge You Need to Help Yourself. Reading, MA: Perseus Books, 1993. A comprehensive guide to coping with lupus medically and in daily life.
The National Institutes of Health posts information about lupus on its website. http://www.nih.gov/niams/healthinfo/slehandout
Further information may be obtained from the website of the Lupus Foundation of America. http://internet-plaza.net/lupus/
"Lupus." Complete Human Diseases and Conditions. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/lupus
"Lupus." Complete Human Diseases and Conditions. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/lupus
"lupus." World Encyclopedia. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/environment/encyclopedias-almanacs-transcripts-and-maps/lupus
"lupus." World Encyclopedia. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/environment/encyclopedias-almanacs-transcripts-and-maps/lupus
Lu·pus / ˈloōpəs/ Astron. a southern constellation (the Wolf), lying partly in the Milky Way between Scorpius and Centaurus. ∎ [as genitive] (Lu·pi / -pī/ ) used with a preceding letter or numeral to designate a star in this constellation: the star Delta Lupi.
"Lupus." The Oxford Pocket Dictionary of Current English. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-0
"Lupus." The Oxford Pocket Dictionary of Current English. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-0
lu·pus / ˈloōpəs/ • n. any of various ulcerous skin diseases, esp. lupus vulgaris or lupus erythematosus. DERIVATIVES: lu·poid / -ˌpoid/ adj. lu·pous / -pəs/ adj.
"lupus." The Oxford Pocket Dictionary of Current English. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-1
"lupus." The Oxford Pocket Dictionary of Current English. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-1
"lupus." The Concise Oxford Dictionary of English Etymology. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-2
"lupus." The Concise Oxford Dictionary of English Etymology. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus-2
"lupus." Oxford Dictionary of Rhymes. . Encyclopedia.com. (October 21, 2016). http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus
"lupus." Oxford Dictionary of Rhymes. . Retrieved October 21, 2016 from Encyclopedia.com: http://www.encyclopedia.com/humanities/dictionaries-thesauruses-pictures-and-press-releases/lupus