systemic lupus erythematosus


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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.
  • 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
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.
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.)
  • 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).

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.

lupus

 [loo´pus]
a name originally given to a destructive type of skin lesion, implying a local degeneration rather than systemic involvement. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. The Latin word lupus means wolf; erythematosus refers to redness. The name lupus erythematosus has been used since the 13th century because physicians thought the shape and color of the skin lesions resembled the bite of a wolf. Currently, there are at least two recognized manifestations of the disease: discoid lupus erythematosus and systemic lupus erythematosus.
chilblain lupus erythematosus a form of discoid lupus erythematosus aggravated by cold, initially resembling chilblains, in which the lesions consist of reddened infiltrated patches on the exposed areas of the body, especially the finger knuckles.
cutaneous lupus erythematosus one of the two main types of lupus erythematosus; it may involve only the skin or may precede involvement of other body systems. It may be chronic (discoid lupus erythematosus); subacute (systemic lupus erythematosus); or acute (characterized by an acute edematous, erythematous eruption, often with systemic exacerbations). The acute form may be the presenting symptom of systemic lupus erythematosus, such as after sun exposure.
discoid lupus erythematosus (DLE) a superficial inflammation of the skin, marked by red macules up to 3 to 4 cm in width, and covered with scanty adherent scales, which extend into spreading follicles that fall off and leave scars. The lesions typically form a butterfly pattern over the bridge of the nose and cheeks, but other areas may be involved, notably the scalp and other areas that are exposed to light.
drug-induced lupus a syndrome closely resembling systemic lupus erythematosus, precipitated by prolonged use of certain drugs, most commonly hydralazine, isoniazid, various anticonvulsants, and procainamide.
lupus erythemato´sus (LE) a group of connective tissue disorders primarily affecting women aged 20 to 40, comprising a spectrum of clinical forms in which cutaneous disease may occur with or without systemic involvement.
lupus per´nio
1. soft, purplish skin lesions on the cheeks, forehead, nose, ears, and digits, frequently associated with bone cysts, which may be the first manifestation of sarcoidosis or occur in the chronic stage of the disease.
systemic lupus erythematosus (SLE) a chronic inflammatory disease, usually febrile and characterized by injury to the skin, joints, kidneys, nervous system, and mucous membranes. It can, however, affect any organ of the body and usually has periods of remissions and exacerbations. (See plate in Dermatology Atlas.)

It was once thought that this was a fairly rare disease, but improved immunologic testing procedures have shown that it is not. It is primarily a disease of women, occurring five to ten times more often in females than in males. Although the peak incidence is between 30 and 40 years of age, the condition has also been diagnosed in the very young and the very old.

SLE is the classic prototype of autoimmune disease of connective tissue. Its etiology is unknown, but the high level of autoantibodies in persons with the condition indicates a defect in the regulatory mechanisms that sustain self-tolerance and prevent the body from attacking its own cells, cell constituents, and proteins. Patients with SLE can have a wide variety of autoantibodies against nuclear and cytoplasmic cellular components. The presence of high levels of antinuclear antibody (ANA) in SLE patients with glomerulonephritis indicates a pathogenic role for that antibody. The antibodies are directed against deoxyribonucleoprotein, DNA, histone, and a soluble non–nucleic acid molecule called Sm antigen.

Factors that appear to contribute to the development of SLE include exposure to sunlight or ultraviolet radiation from sunlamps, a genetic predisposition to the disease, certain drugs, viral infections, and hormonal influences.

Clinical manifestations of SLE are confusingly diverse owing to the involvement of connective tissue throughout the body. Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis. The detection of ANA by microscopic immunofluorescence is supportive evidence for the presence of SLE.

Either glomerulonephritis, which is usually mild, or cardiovascular manifestations such as myocarditis, endocarditis, or pericarditis, are found in about half the patients with SLE. Pulmonary disease, especially pleurisy, is also relatively common, as are gastrointestinal disturbances and lymph node involvement. Organic neurologic disturbances produce behavioral aberrations and frank psychosis in some patients; in a few others, there are peripheral neuropathies, motor weakness, and diplopia.

Supportive measures are used to prevent or minimize acute relapses and exacerbations of symptoms. The patient is instructed to avoid exposure to sunlight and ultraviolet radiation from other sources, blood transfusions, penicillin, and the sulfonamides. Active disease is treated with topical steroids, nonsteroidal antiinflammatory drugs for fever and joint pain, corticosteroids, and immunosuppressants. The goal of drug therapy is suppression of the immune system. Treatment of specific manifestations of SLE is aimed at prevention of complications. Physical therapy may be required to alleviate muscle weakness and prevent orthopedic deformities.
lupus vulga´ris the most common and severe form of tuberculosis of the skin, most often affecting the face, with formation of reddish brown patches of nodules in the corium, which progressively spread peripherally with central atrophy, causing ulceration and scarring and destruction of cartilage in involved sites.

sys·tem·ic lu·pus er·y·the·ma·to·sus (SLE),

[MIM*152700]
an inflammatory connective tissue disease with variable features, frequently including fever, weakness and fatigability, joint pains or arthritis resembling rheumatoid arthritis, diffuse erythematous skin lesions on the face, neck, or upper extremities, with liquefaction degeneration of the basal layer and epidermal atrophy, lymphadenopathy, pleurisy or pericarditis, glomerular lesions, anemia, hyperglobulinemia, and a positive LE cell test result, with serum antibodies to nuclear protein and sometimes to double-stranded DNA and other substances.

systemic lupus erythematosus

(ĕr′ə-thē′mə-tō′sĭs)
n. Abbr. SLE
An inflammatory autoimmune disease that affects multiple organ systems, usually characterized by fatigue, fever, skin lesions, joint pain and swelling, and anemia, and often affecting the kidneys and various other organs. It is much more common in women than in men.

systemic lupus erythematosus (SLE)

a chronic inflammatory disease affecting many systems of the body. It is an example of a collagen disease. The pathophysiological characteristics of the disease include severe vasculitis, renal involvement, and lesions of the skin and nervous system. The primary cause of the disease has not been determined; viral infection or dysfunction of the immune system has been suggested. Adverse reaction to certain drugs also may cause a lupuslike syndrome. Four times more women than men have SLE. Also called disseminated lupus erythematosus. See also discoid lupus erythematosus.
observations The initial manifestation is often arthritis. An erythematous rash ("butterfly rash") over the nose and malar eminences, weakness, fatigue, and weight loss also are frequently seen early in the disease. Photosensitivity, fever, skin lesions on the neck, and alopecia where the skin lesions extend beyond the hairline may occur. The skin lesions may spread to the mucous membranes and other tissues of the body. They do not ulcerate but cause degeneration of the affected tissues. Depending on the organs involved, the patient also may have glomerulonephritis, pleuritis, pericarditis, peritonitis, neuritis, or anemia. Renal failure and severe neurological abnormalities are among the most serious manifestations of the disease. Diagnosis of SLE is made by subjective and objective findings based on physical examination and laboratory findings, including antinuclear antibody in the cerebrospinal fluid and a positive lupus erythematosus cell reaction in a lupus erythematosus preparation. Other laboratory examinations may be useful, depending on the organs, tissues, and systems affected by the disease.
interventions In many cases SLE may be controlled with corticosteroid medication administered systemically. Care and treatment vary with the severity and nature of the disease and the body systems that are affected. Topical steroids may be applied to the rash. Salicylates may be given to alleviate pain and swelling in the joints. Fatigue and stress are prevented, and all body surfaces are protected from direct sunlight. Antimalarial drugs are sometimes given to treat cutaneous lesions, but retinal damage may occur with prolonged use.
nursing considerations The timing, dosage, side effects, and toxic reactions to the medications are explained before discharge. The steroids must be taken exactly as prescribed, and, in the event that the patient cannot take them, the doctor is to be consulted promptly. The patient should carry an identification card bearing his or her diagnosis, a list of all medications and their dosage, and the doctor's name and telephone number. As in any disease marked by chronic remission and exacerbation of many distressing symptoms, the patient may require extensive emotional and psychological support.
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Classic butterfly rash of systemic lupus erythematosus

sys·tem·ic lu·pus er·y·the·ma·to·sus

(SLE) (sis-tem'ik lū'pŭs ĕr-ith'ĕ-mă-tō'sŭs)
An inflammatory connective tissue disease with variable features including fever; weakness and fatigability; joint pains or arthritis resembling rheumatoid arthritis; diffuse erythematous skin lesions on the face, neck, or upper extremities; lymphadenopathy; pleurisy or pericarditis; glomerular lesions; anemia; hyperglobulinemia; and a positive LE cell test result, with serum antibodies to double-stranded DNA and acidic nuclear protein (Sm).
Synonym(s): disseminated lupus erythematosus.
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SYSTEMIC LUPUS ERYTHEMATOSUS

systemic lupus erythematosus

Abbreviation: SLE
A chronic autoimmune inflammatory disease of connective tissue involving multiple organ systems and marked by periodic acute episodes. Its name is derived from the characteristic erythematous butterfly rash over the nose and cheeks, which resembles a wolf's snout, although this is present in less than 50% of patients. The disease is most prevalent in women (ratio of 8:1 women:men) of childbearing age (ratio of 15:1). Although it occurs worldwide, it is most prevalent among black and Asian peoples.

Etiology and Pathology

SLE is classified as an autoimmune disease in which the body seems to be unable to maintain normal mechanisms of tolerance to autoantigens. Activation of T helper cells and B cells results in the production of autoantibodies that attack antigens in the cytoplasm and nucleus of cells and on the surface of blood cells. The exact cause of SLE is unknown: genetic defects, hormonal changes, infection, physical or mental stress, some drugs, immunizations, and environmental triggers (sunlight, UV light exposure) are possible predisposing factors. See: autoimmune disease; glomerulonephritis

Autoantibodies can react with autoantigens to form immune complexes in such large numbers that they cannot be completely excreted; the immune complexes may precipitate within blood vessels, producing inflammation at the site and disrupting the flow of blood and oxygen to tissues. These deposits are particularly damaging in the glomeruli. Autoantibodies also promote the destruction of cells by stimulating neutrophil and macrophage phagocytic activity, which increases cell destruction from trauma, infection, or drugs.

Diagnosis

In 1997, revised criteria for diagnosis of SLE were established. The diagnosis can be made if four or more of the following criteria are present, either at one time or sequentially: (1) butterfly rash; (2) raised, scaly discoid skin lesions; (3) abnormal titer of antinuclear antibodies seen by immunofluorescence; (4) other autoantibodies (anti-Sm; serological tests for syphilis); (5) pleuritis or pericarditis (together referred to as “serositis”); (6) hemolytic anemia, leukopenia (white blood cell count less than 4,000 mm3), lymphopenia (lymphocyte count less than 1,500/mm3), or thrombocytopenia of less than 100,000/mm3; (7) oral or nasopharyngeal ulcers; (8) nonerosive arthritis; (9) psychosis or seizures without other clear cause; (10) photosensitivity skin rash; and (11) proteinuria greater than 0.5 g/day or cellular casts in the urine.

Some drugs can cause a lupus-like syndrome; the most common of these are procainamide, isoniazid, and hydralazine. See: drug-induced systemic lupus erythematosus

Symptoms

The onset of the disease may be acute or insidious. Patients have a wide variety of clinical symptoms, signs, and laboratory findings, but anemia, thrombocytopenia, polyarthritis, (polyarthralgia) skin rashes, glomerulonephritis, fever, malaise, weight loss, fatigue, and low blood levels of complement are the most common. Other signs include pleuritis, pericarditis, myocarditis, neurological changes including behavioral changes and seizure activity (neural lupus), gastrointestinal ulcerations, Raynaud's phenomenon (present in about 20% of patients), and other problems caused by inflammatory changes of the blood vessels or connective tissue. Most patients are prone to infection.

Treatment

No cure for SLE exists, and complete remission is rare. About 25% of patients have mild disease, demonstrating only minor skin and hematological signs, and can be treated with nonsteroidal anti-inflammatory drugs for their arthritis symptoms and topical treatment (sometimes with corticosteroid creams) for skin lesions. Rashes may respond to antimalarials, e.g., hydroxychloroquine, but patients must be observed closely for the possibility of drug-induced retinal damage. Other treatments for skin rash include quinacrine, retinoids, and dapsone. Life-threatening and severely disabling conditions should be treated with high doses of corticosteroids and supplemental calcium to minimize osteoporosis, which may be an undesired side effect of long-term glucocorticoid use. Immunosuppressive drugs are used for severe exacerbations and to reduce steroid dosage.

Prognosis

The prognosis depends on which organ systems are involved, how severely they are damaged, and how rapidly the disease progresses. Ten-year survival rates are high (80%). Renal failure and infections are the most common causes of death.

Patient care

Patient education related to the disease, diagnostic procedures, and treatment is essential in lupus, as in any chronic disease. Ongoing assessment is carried out to assess flares of the illness. The purpose, proper dosage, use, and side effects of drugs is taught. Patients need emotional support to help cope with changes in appearance. Patients should be taught to wear clothing and hats that block direct sunlight, use a sunscreen with a 15 or higher protection factor, and to maintain a diet appropriate for their renal functional status. The health care professional should help establish a regimen for adequate relief of both the musculoskeletal pain and chronic fatigue experienced by most patients, encouraging adequate rest. Heat packs relieve joint stiffness and pain, and regular gentle exercise helps to maintain full range of motion. Physical and occupational therapy consultations are provided as appropriate. Additional support and teaching depend on the organ system most affected by the disease. If the female patient of childbearing age has no renal or neurologic impairment, she can have a safe, successful pregnancy if desired. Over time, patients with severe progressive disease need assistance in coping with chronic illness and the possibility of mortality. Referrals to the Lupus Foundation of America (202-349-1155; www.lupus.org) and the Arthritis Foundation (800-283-7800; www.arthritis.org) are helpful.

See: illustration
See also: lupus

systemic lupus erythematosus

The form of LUPUS ERYTHEMATOSUS that affects the body generally.

lupus

generic term for systemic and discoid lupus erythematosus
  • discoid lupus erythematosus; DLE localized systemic lupus erythematosus (SLE), characterized by similar skin lesions, but without marked systemic effects; may later progress to SLE

  • systemic lupus erythematosus; SLE; lupus autoimmune, inflammatory, connective tissue disease, characterized variably by fever, weakness and fatigability, joint pain and/or rheumatoid-like arthritis, severe Raynaud's phenomenon, diffuse erythematous vasculitis of sun-exposed skin (facial 'butterfly' rash), red nails/lunulae, epidermal atrophy (± ulceration), lymphadenopathy, pleurisy, pericarditis, glomerulonephritis, anaemia, hyperglobinaemia; more common in younger women, and women of Black African ancestry

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.
Enlarge picture
Systemic lupus erythematosus (SLE).

scleritis 

Inflammation of the sclera, which in its severe necrotizing or in the posterior type may cause sight-threatening complications such as keratitis, uveitis, angle-closure glaucoma or optic neuropathy. It affects females more commonly than males in the fourth to sixth decades of life. Like episcleritis it has a tendency to recur. It is characterized by pain, which can be severe, redness, tearing and some patients may develop nodules (nodular scleritis). It is often associated with a systemic disease (e.g. rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, lupus erythematosus, ankylosing spondylitis, syphilis, herpes zoster). It can involve part of the sclera, e.g. anterior scleritis (which is the most common, and it is classified as diffuse non-necrotizing or nodular non-necrotizing) or posterior scleritis. Treatment includes topical and systemic steroids and immunosuppressive drugs for very severe cases. See acute stromal keratitis; Brown's superior oblique tendon sheath syndrome.
necrotizing scleritis The most severe form of scleritis, much less common than the other types. About half the patients have one of the following diseases: rheumatoid arthritis, Wegener's granulomatosis, polyarteritis nodosa, systemic lupus erythematosus, or herpes zoster. It is characterized by pain, and white, avascular areas next to damaged areas through which one can see the brown colour of the underlying uveal tissue, and to congested areas of the sclera. In most cases visual acuity is decreased. The necrosis gradually spreads around the globe. Treatment typically consists of topical steroids, immunosuppressive agents and occasionally surgery to repair scleral or corneal perforation. See keratolysis; scleromalacia.
scleritis necroticans See scleromalacia.
posterior scleritis Inflammation of the sclera involving the posterior segment of the eye. The condition is often associated with a systemic disease (e.g. rheumatoid arthritis). It is characterized by pain and reduced visual acuity. The severity of the visual impairment depends on the involved tissue and its location. Signs include eyelid oedema, proptosis, limitation of ocular movements and, if anterior scleritis is present, redness. The ocular fundus may present disc swelling, choroidal folds, macular oedema and serous retinal detachment. Treatment consists mainly of systemic steroids and immunosuppressive agents. See choroidal folds.

sys·tem·ic lu·pus er·y·the·ma·to·sus

(SLE) (sis-tem'ik lū'pŭs ĕr-ith'ĕ-mă-tō'sŭs) [MIM*152700]
Inflammatory connective tissue disease with variable features, frequently including fever, weakness and fatigability, joint pains or arthritis resembling rheumatoid arthritis, and diffuse erythematous skin lesions on the face, neck, or upper limbs.

systemic lupus erythematosus (SLE) (sistem´ik loo´pus er´ə-them´ətō´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.

systemic

pertaining to or affecting the body as a whole.

systemic inflammatory response (SIR)
a specific condition recognized in critical care medicine, characterized by febrile response, increased heart and respiratory rates, and leukocytosis.
systemic lupus erythematosus
systemic neuroaxial dystrophy
systemic therapy
treatment with a drug that is absorbed into the bloodstream and distributed to all parts of the body except where there is a specific barrier.

Patient discussion about systemic lupus erythematosus

Q. Is a rash a symptom of lupus? My Sister has lupus for several years now. I recently developed a rash on my face. Is this a symptom of lupus? Could I have also been infected with this disease?

A. Lupus has not been proven to be hereditary. Therefore, the fact your sister has lupus shouldn't cause you to beleive you too will develop it. Also a rash is not enough to diagnose lupus. Physicians have to gather information from a variety of sources: past medical history, lab tests and current symptoms. They use a list of 11 criteria to help diagnose SLE. A person needs to satisfy at least 4 out of the 11 criteria before the diagnosis can be pinpointed. Some criteria, such as a biopsy diagnosis of kidney lupus, can carry more weight.

More discussions about systemic lupus erythematosus
References in periodicals archive ?
Clinical manifestations in 32 children with systemic lupus erythematosus Clinical manifestations Present at any time n % Constitutional 30 93.
Causes of mortality among inpatients with systemic lupus erythematosus in a tertiary care hospital in North India over a 10-year period.
The effect of hydroxychloroquine therapy on serum levels of immunoregulatory molecules in patients with systemic lupus erythematosus.
For systemic lupus erythematosus (SLE) induced by hydralazine, musculoskeletal symptoms are the most common clinical manifestation.
A review of the Systemic Lupus Erythematosus products under development by companies and universities/research institutes based on information derived from company and industry-specific sources.
Neuropsychiatric systemic lupus erythematosus has been considered in SLE patients with neurological and psychiatric disorders, but without other identified causes.
Collaboration to focus on research and development of antibodies for the treatment of systemic lupus erythematosus (SLE)
These findings and the team's associated research in humans may lead to new treatments for this serious disease, a severe form of systemic lupus erythematosus (SLE) that affects the kidneys and is difficult to treat.

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