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Systemic Lupus Erythematosus

What is systemic lupus erythematosus (SLE)?

Some call it the "great imitator," because its symptoms vary so widely that it is often mistaken for other disorders. Systemic lupus erythematosus, often referred to simply as lupus, is a serious disease affecting the joints, kidneys, and skin.

Lupus erythematosus is a chronic inflammatory disorder of the connective tissues that appears in two forms: discoid lupus erythematosus, which affects only the skin, and systemic lupus erythematosus (SLE), which affects multiple organ systems as well as the skin and can be fatal. SLE is characterized by recurring remissions and exacerbations, which are especially common during the spring and summer. The prognosis improves with early detection and treatment but remains poor for patients who develop cardiovascular, renal or neurologic complications. or severe bacterial infections.

SLE is more common in women than in men. Approximately 90% of SLE cases occur in women. SLE is onset is typically during a woman's reproductive years beginning as early as age 14. Black women are three times more affected than White women. Asian women are also at increased risk.

What causes Systemic Lupus Erythematosus?

Auto immunity is believed to be the prime mechanism involved in SLE. The body produces antibodies against components of its own cells. such as the antinuclear antibody (ANA). and immune complex disease follows. Women with SLE may produce antibodies against many different tissue components, such as red blood cells (RBCs), neutropbils, platelets,lymphocytes, or almost any organ or tissue in the body.

Although the exact cause of SLE remains a mystery, available evidence points to interrelated immunologic, environmental, hormonal, and genetic factors, which may include:

  • physical or mental stress
  • streptococcal or viral infections
  • exposure to sunlight or ultraviolet light
  • immunization
  • pregnancy
  • abnormal estrogen metabolism
  • treatment with certain drugs, such as procainamide (Pronestyl), hydralazine (Apresoline), anticonvulsants and, less commonly, penicillin's, sulfa drugs, and hormonal contraceptives.

Signs and symptoms of Systemic Lupus Erythematosus

The onset of SLE may be acute or insidious and produces no characteristic clinical pattern.

Although SLE may involve any organ system, signs and symptoms all relate to tissue injury and subsequent inflammation and necrosis resulting from the invasion by immune complexes. They commonly include:

  • fever
  • weight loss
  • malaise
  • fatigue
  • rashes (butterfly pattern)
  • polyarthralgia.
Systemic Lupus Erythematosus

Additional signs and symptoms may include:

  • joint involvement, similar to rheumatoid arthritis (although the arthritis of lupus is usually nonerosive)
  • skin lesions, most commonly an erythematous rash in areas exposed to light (the classic butterfly rash over the nose and cheeks occurs in less than 50% of patients) or a scaly, popular rash (mimics psoriasis), especially in sun-exposed areas
  • vasculitis (especially in the digits). possibly leading to infarctive lesions, necrotic leg ulcers or digital gangrene
  • Raynaud's phenomenon (about 20% of patients)
  • patchy alopecia and painless ulcers of the mucous membranes
  • pulmonary abnormalities, such as pleurisy, pleural effusions, pneumonitis, pulmonary hypertension and, rarely, pulmonary hemorrhage
  • cardiac involvement, such as pericarditis, myocarditis, endocarditis, and early coronary atherosclerosis
  • microscopic hematuria, pyuria, and urine sediment with cellular casts due to glomerulonephritis, possibly progressing to kidney failure (particularly when untreated)
  • urinary tract infections, possibly due to heightened susceptibility to infection
  • seizure disorders and mental dysfunction
  • central nervous system (CNS) involvement, such as emotional instability, psychosis, and organic brain syndrome
  • headaches, irritability, and depression (common).

Classic symptoms of SLE include:

  • aching, malaise,fatigue
  • low-grade or spiking fever and chills
  • anorexia and weight loss
  • lymph node enlargement (diffuse or local; non tender)
  • abdominal pain
  • nausea, vomiting, diarrhea, constipation
  • irregular menstrual periods or amenorrhea during the active phase of SLE.

Possible complications of SLE include concomitant infections, urinary tract infections, renal failure, and osteonecrosis of hip from long-term steroid use.

 
The 10-year survival rate for lupus patients exceeds 85%. People with severe involvement of the brain, lungs, heart and kidney have the worst prognosis in terms of overall survival and disability.
Diagnosis tests information

Diagnostic test results that may indicate SLE include:

  • complete blood count with differential showing anemia and a decreased white blood cell (WBC) count
  • decreased platelet count
  • elevated erythrocyte sedimentation rate
  • serum electrophoresis showing hyperganunaglobulinemia.

Other diagnostic tests used to aid diagnosis of SLE include:

  • ANA and lupus erythematosus cell tests, which show positive results in active SLE
  • anti-double-stranded deoxyribonucleic acid antibody (anti-dsDNA), which is the most specific test for SLE and correlates with disease activity, especially renal involvement. to help monitor response to therapy (possibly low or absent in remission)
  • urine studies, which may show RBCs and WBCs, urine casts and sediment, and significant protein loss (more than 0.5 g/24 hours)
  • serum complement blood studies, which may show decreased serum complement (C3 and C4) levels indicating active disease
  • chest X-ray, which may show pleurisy or lupus pneumonitis
  • electrocardiography, which may show a conduction defect with cardiac involvement or pericarditis
  • kidney biopsy to determine disease stage and extent of renal involvement
  • lupus anticoagulant and anticardiolipin tests, which may be positive in some patients (usually in patients prone to antiphospholipid syndrome of thrombosis, abortion, and thrombocytopenia).

Treatment of Systemic Lupus Erythematosus

Treatment for SLE may include:

  • nonsteroidal anti-inflammatory compounds, including aspirin, to control arthritis symptoms
  • topical corticosteroid creams such as hydrocortisone buteprate (Acticort) or triamcinolone (Aristocort) for acute skin lesions
  • intralesional corticosteroids or antimalarials such as bydroxychloroquine sulfate (Plaquenil) to treat refractory skin lesions
  • systemic corticosteroids to reduce systemic symptoms of SLE for acute, generalized exacerbations or for serious disease related to vital organ systems, such as pleuritis, pericarditis, lupus nephritis, vasculitis, and CNS involvement
  • high-dose steroids and cytotoxic therapy such as cyclophosphamide (Cytoxan) to treat diffuse proliferative glomerulonephritis
  • dialysis or kidney transplant for renal failure
  • antihypertensive drugs and dietary changes to minimize effects of renal involvement.

Special considerations and Prevention

Careful assessment, supportive measures, emotional support, and patient education are all important parts of the care plan for patients with SLE:

  • Watch for classic symptoms, such as joint pain or stiffness, weakness, fever, fatigue, and chills. Observe for dyspnea, chest pain, and edema of the extremities. Note the size, type, and location of skin lesions. Check urine for hematuria, scalp for hair loss, and skin and mucous membranes for petechiae, bleeding, ulceration, pallor, and bruising.
  • Provide a balanced diet Renal involvement may mandate a low sodium, low-protein diet.
  • Urge the patient to get plenty of rest. Schedule diagnostic tests and procedures to allow adequate rest. Explain all tests and procedures. Tell the patient that several blood samples are needed initially, then periodically, to monitor progress.
  • Explain the expected benefit of prescribed medications. Watch for adverse effects, especially when the patient is taking high doses of corticosteroids.
  • Advise the patient receiving cyclophosphamide to maintain adequate hydration. If prescribed, give mesna to prevent hemorrhagic cystitis and ondansetron to prevent nausea and vomiting.
  • The best way to control lupus is to be very careful to take all your medications as prescribed, visit your physician regularly, and learn as much as you can about lupus, your medications, and your progress. Get involved in your care. Don't take a back seat.
  • Monitor vital signs, intake and out put, weight, and laboratory reports. Check pulse rates and observe for orthopnea. Check stools and GI secretions for blood.
  • Observe for hypertension, weight gain, and other signs of renal involvement.
  • Assess for signs of neurologic damage, such as personality change, paranoid or psychotic behavior, ptosis, and diplopia. Take seizure precautions. If Raynaud's phenomenon is present, warm and protect the patient's hands and feet.
  • Offer cosmetic tips, such as suggesting the use of hypo allergenic makeup and referring the patient to a hairdresser who specializes in scalp disorders.
  • Advise the patient to purchase medications in quantity, if possible. Warn against "miracle" drugs for relief of arthritis symptoms.


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