Lupus erythematosus
Lupus erythematosus: Excerpt from Professional Guide to Diseases (Eighth Edition)
A chronic inflammatory disorder of the connective tissues, lupus erythematosus appears in two forms. Discoid lupus erythematosus affects only the skin. (See Discoid lupus erythematosus.) Systemic lupus erythematosus (SLE) affects multiple organ systems as well as the skin and can be fatal. Like rheumatoid arthritis, SLE is characterized by recurring remissions and exacerbations, 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.
Causes and incidence
The exact cause of SLE remains a mystery, but evidence points to interrelated immunologic, environmental, hormonal, and genetic factors. Autoimmunity is thought to be the prime causative mechanism. In autoimmunity, the body produces antibodies against its own cells such as the antinuclear antibody. The formed antigen-antibody complexes can suppress the body’s normal immunity and damage tissues. Patients with SLE produce antibodies against many different tissue components, such as red blood cells (RBCs), neutrophils, platelets, lymphocytes, or almost any organ or tissue in the body.
Certain predisposing factors may make a person susceptible to SLE. Physical or mental stress, streptococcal or viral infections, exposure to sunlight or ultraviolet light, immunization, pregnancy, and abnormal estrogen metabolism may all affect this disease’s development.
SLE may also be triggered or aggravated by treatment with certain drugs — for example, procainamide, hydralazine, anticonvulsants and, less commonly, penicillins, sulfa drugs, and hormonal contraceptives.
SLE strikes 8 times more females than men, increasing to 15 times more during childbearing years. It occurs worldwide but is most prevalent among Asians and Blacks.
Signs and symptoms
The onset of SLE may be acute or insidious and produces no characteristic clinical pattern. However, its symptoms commonly include fever, weight loss, malaise, and fatigue as well as rashes and polyarthralgia. SLE may involve every organ system. In 90% of patients, joint involvement is similar to that in rheumatoid arthritis. Skin lesions are most commonly erythematous rashes in areas exposed to light. The classic butterfly rash over the nose and cheeks occurs in fewer than 50% of the patients. (See < /span>Butterfly rash.) Ultraviolet rays often provoke or aggravate skin eruptions. Vasculitis can develop (especially in the digits), possibly leading to infarctive lesions, necrotic leg ulcers, or digital gangrene. Raynaud’s phenomenon appears in about 20% of patients. Patchy alopecia and painless ulcers of the mucous membranes are common.
Constitutional symptoms of SLE include aching, malaise, fatigue, low-grade or spiking fever, chills, anorexia, and weight loss. Lymph node enlargement (diffuse or local, and nontender), abdominal pain, nausea, vomiting, diarrhea, and constipation may occur. Females may experience irregular menstrual periods or amenorrhea during the active phase of SLE.
About 50% of SLE patients develop signs of cardiopulmonary abnormalities, such as pleuritis, pericarditis, and dyspnea. Myocarditis, endocarditis, tachycardia, parenchymal infiltrates, and pneumonitis may occur. Renal effects may include hematuria, proteinuria, urine sediment, and cellular casts, which may progress to total kidney failure. Urinary tract infections may result from heightened susceptibility to infection. Seizure disorders and mental dysfunction may indicate neurologic damage. Central nervous system (CNS) involvement may produce emotional instability, psychosis, and organic mental syndrome. Headaches, irritability, and depression are common. (See Signs of systemic lupus erythematosus.)
Diagnosis
Diagnostic tests for patients with SLE include a complete blood count with differential (for signs of anemia and decreased white blood cell [WBC] count); platelet count (may be decreased); erythrocyte sedimentation rate (commonly elevated); and serum electrophoresis (may show hypergammaglobulinemia).
Specific tests for SLE include:
❑ antinuclear antibody panel, including anti-deoxyribonucleic acid (DNA) and anti-Smith antibodies — generally positive for lupus alone. (Because the anti-DNA test is rarely positive in other conditions, it’s the most specific test for SLE. However, if the patient is in remission, anti-DNA may be reduced or absent [correlates with disease activity, especially renal involvement, and helps monitor response to therapy]. Other tests may be performed as needed to rule out other disorders.)
❑ urine studies — may show RBCs and WBCs, urine casts and sediment, and significant protein loss (more than 0.5 g/24 hours)
❑ blood studies — decreased serum complement (C3 and C4) levels indicate active disease
❑ chest X-ray — may show pleurisy or lupus pneumonitis
❑ electrocardiogram — may show conduction defect with cardiac involvement or pericarditis
❑ kidney biopsy — determines disease stage and extent of renal involvement.
Some patients show a positive lupus anticoagulant test and a positive anticardiolipin test. Such patients are prone to antiphospholipid syndrome (thrombosis and thrombocytopenia).
Treatment
Patients with mild disease require little or no medication. Nonsteroidal anti-inflammatory drugs, including aspirin, control arthritis symptoms in many patients. Skin lesions need topical treatment. Corticosteroid creams are recommended for acute lesions.
Refractory skin lesions are treated with intralesional corticosteroids or antimalarials such as hydroxychloroquine. Because hydroxychloroquine can cause retinal damage, such treatment requires ophthalmologic examination every 6 months.
Corticosteroids remain the treatment of choice for 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. Initial doses equivalent to 60 mg or more of prednisone often bring noticeable improvement within 48 hours. As soon as symptoms are under control, steroid dosage is tapered slowly. (Rising serum complement levels and decreasing anti-DNA titers indicate patient response.) Diffuse proliferative glomerulonephritis, a major complication of SLE, requires treatment with large doses of steroids. If renal failure occurs, dialysis or kidney transplant may be necessary. In some patients, cytotoxic drugs may delay or prevent deteriorating renal status. Antihypertensive drugs and dietary changes may also be warranted in renal disease.
The photosensitive patient should wear protective clothing (hat, sunglasses, long sleeves, and slacks) and use a screening agent, with a sun protection factor of at least 15, when outdoors. Because SLE usually strikes females of childbearing age, questions about pregnancy commonly arise. Available evidence indicates that a woman with SLE can have a safe, successful pregnancy if she has no serious renal or neurologic impairment.
Special considerations
Careful assessment, supportive measures, emotional support, and patient education are all important parts of the care plan for patients with SLE.
❑ Watch for constitutional symptoms: joint pain or stiffness, weakness, fever, fatigue, and chills. Observe for dyspnea, chest pain, and any 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.
❑ Apply heat packs to relieve joint pain and stiffness. Encourage regular exercise to maintain full range of motion (ROM) and prevent contractures. Teach ROM exercises as well as body alignment and postural techniques. Arrange for physical therapy and occupational counseling as appropriate.
❑ 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.
❑ Monitor vital signs, intake and output, 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: personality change, paranoid or psychotic behavior, ptosis, or 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 hypoallergenic makeup and refer 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.
❑ Refer the patient to the Lupus Foundation of America and the Arthritis Foundation as needed.
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Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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