systemic lupus erythematosus

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.

(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.

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lupus /lu·pus/ (loo´pus) any of a group of skin diseases in which the lesions are characteristically eroded.

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chilblain lupus erythematosus  a form due to cold-induced microvascular injury, aggravated by cold; the lesions initially resemble chilblains but eventually assume the form of discoid lupus erythematosus.

cutaneous lupus erythematosus  one of the two main forms of lupus erythematosus, in which the skin may be either the only or the first organ or system involved. It may be chronic (discoid l. erythematosus), subacute (systemic l. erythematosus), or acute (characterized by an acute, edematous, erythematous eruption).

discoid lupus erythematosus  (DLE) a chronic form of cutaneous lupus erythematosus marked by red macules covered with scanty adherent scales that fall off and leave scars; lesions typically form a butterfly pattern over the bridge of the nose and cheeks, but other areas may be involved.

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 chronic connective tissue diseases manifested in two main types: cutaneous l. erythematosus and systemic l. erythematosus.

lupus erythemato´sus profun´dus  a form of cutaneous lupus erythematosus in which deep brawny indurations or subcutaneous nodules occur under normal or, less often, involved skin; the overlying skin may be erythematous, atrophic, and ulcerated and on healing may leave a depressed scar.

lupus erythemato´sus tu´midus  a variant of discoid or systemic lupus erythematosus in which the lesions are raised reddish purple or brown plaques.

hypertrophic lupus erythematosus  a form of discoid lupus erythematosus characterized by verrucous hyperkeratotic lesions.

lupus hypertro´phicus 

1. a variant of lupus vulgaris in which the lesions consist of a warty vegetative growth, often crusted or slightly exudative, usually occurring on moist areas near body orifices.

2. hypertrophic l. erythematosus.

lupus milia´ris dissemina´tus fa´ciei  a form marked by multiple, discrete, superficial nodules on the face, particularly on the eyelids, upper lip, chin, and nares.

neonatal lupus  a rash resembling discoid lupus erythematosus, sometimes with systemic abnormalities such as heart block or hepatosplenomegaly, in infants of mothers with systemic lupus erythematosus; it is usually benign and self-limited.

lupus per´nio 

1. a cutaneous manifestation of sarcoidosis consisting of violaceous smooth shiny plaques on the ears, forehead, nose, and digits, frequently associated with bone cysts.

2. chilblain l. erythematosus.

systemic lupus erythematosus  (SLE) a chronic generalized connective tissue disorder, ranging from mild to fulminating, marked by skin eruptions, arthralgia, arthritis, leukopenia, anemia, visceral lesions, neurologic manifestations, lymphadenopathy, fever, and other constitutional symptoms. Typically, there are many abnormal immunologic phenomena, including hypergammaglobulinemia and hypocomplementemia, deposition of antigen-antibody complexes, and the presence of antinuclear antibodies and LE cells.

lupus vulga´ris  the most common and severe form of tuberculosis of the skin, most often affecting the face, with formation of red-brown patches of nodules in the dermis that progressively spread peripherally with central atrophy, causing ulceration and scarring and destruction of cartilage in involved sites.

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systemic lupus er·y·the·ma·to·sus (r-thm-tss, -thm-)

n.

Abbr. SLE A chronic multisystemic inflammatory disease with variable features, frequently including fever, weakness and fatigability, joint pains or arthritis, skin lesions on the face, neck, or upper extremities, and often affecting the kidneys, spleen, and various other organs. Also called disseminated lupus erythematosus.

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systemic lupus erythematosus (SLE),

a chronic inflammatory disease affecting many systems of the body. It is an example of a collagen disease. The pathophysiologic 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 neurologic 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 psychologic support.

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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.

Systemic lupus erythematosus (SLE).

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

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systemic

pertaining to or affecting the body as a whole.

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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

see lupus erythematosus.

systemic neuroaxial dystrophy

see 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.

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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.

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systemic lupus erythematosus

Disseminated LE, lupus, lupus erythematosus Rheumatology An idiopathic multisystem collagen vascular (connective tissue) disorder Epidemiology Affects ±40/105–North America, Europe, blacks/hispanics > whites, ♀:♂ ratio = 3:1; 80% onset during childbearing yrs Clinical Vasculitis, serositis, synovitis, cerebral, renal, skin involvement Cause of death Sepsis 40%, CVS 20%, CNS 10%, renal 10%, etc 20%. See Anticardiolipin antibody, Antinuclear antibody, Butterfly rash.

Systemic lupus erythematosus–1982 revised criteria, rerevised in 1997

1. Malar rash Fixed erythema, in particular over the malar eminences

2. Discoid rash Raised erythematous patches with adherent hyperkeratotic scaling; atrophic scarring in some old lesions

3. Photosensitivity Unusual skin rashes in response to sunlight

4. Oral ulcers Oral or nasopharyngeal ulcers

5. Arthritis Nonerosive arthritis of 2+ joints, accompanied by tenderness, swelling, or effusions

6. Serositis  

a. Pleuritis OR

b. Pericarditis

7. Renal disease  

a. Persistent proteinuria > 0.5 g/day OR

b. Cellular casts

8. Neurologic disorder  

a. Seizures without substance use or medical disease OR

b. Psychosis in absence of substance use or medical disease

9. Hematologic disorder  

a. Hemolytic anemia OR

b. Leukopenia OR

c. Lymphocytopenia

d. Thrombocytopenia

10. Immunologic disorder  

a. Positive LE cell prep DELETED IN 1997

b. Anti-DNA antibody

c. Anti-Sm antibody

d. False positive serological test for syphilis BECOMES

Positive finding of antiphospholipid antibodies based on

 1) an abnormal serum level of IgG or IgM anticardiolipin antibodies,

 2) a positive test result for lupus anticoagulant using a standard method, or

3) a false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test. Standard methods should be used in testing for the presence of antiphospholipid

11. Antinuclear antibody Abnormal titers of ANA in absence of drugs known to be associated with drug-induced LE www.rheumatology.org/research/classification/1982SLEupdate.html

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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. A rash could be a symptom of cutaneous lupus. The symptoms of cutaneous lupus may include a variety of different looking skin rashes, photosensitivity (where exposure to ultra-violet light triggers a rash), and sometimes ulcers on the inside of the nose or mouth. There are a variety of ways that cutaneous lupus rashes can appear. The distinctive rash is called the "butterfly rash," which is a rash that extends across the cheeks of the face and the bridge of the nose.