Lupus erythematosus
Lupus erythematosus: Excerpt from Handbook of Diseases
A chronic inflammatory disorder of the connective tissues, lupus erythematosus 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. Like rheumatoid arthritis, SLE is characterized by recurring remissions and exacerbations, which are especially common during spring and summer.
SLE strikes 8 times more women than men, increasing to 15 times among women of childbearing years. It occurs worldwide, but is most prevalent among Asians and blacks. The prognosis improves with early detection and treatment, but remains poor for patients who develop cardiovascular, renal, or neurologic complications or severe bacterial infections.
gender influence A report by the Centers for Disease Control and Prevention showed a 60% increase in deaths over a 20-year period from SLE. Women were five times more likely to die of complications than men. Nine out of every 10 patients affected with the disease are women, with the highest prevalence in African-Americans, Hispanics, Asians, and Native Americans in the United States. Research on this disorder is ongoing.
Causes
The exact cause of SLE remains a mystery, but available evidence points to interrelated immunologic, environmental, hormonal, and genetic factors.
Immune dysregulation
Immune dysregulation, in the form of autoimmunity, is thought to be the prime causative mechanism. With autoimmunity, the body produces antibodies against components of its own cells, such as the antinuclear antibody (ANA). The formed antigen-antibody complexes can activate the body’s immunity and damage tissues. One significant feature in patients with SLE is their ability to produce antibodies against many different tissue components, such as red blood cells, neutrophils, platelets, lymphocytes, or almost any organ or tissue in the body.
Predisposing factors
Physical or mental stress, streptococcal or viral infections, exposure to sunlight or ultraviolet light, immunization, pregnancy, and abnormal estrogen metabolism may all affect the development of this disease in a genetically susceptible individual.
SLE also may be triggered or aggravated by treatment with certain drugs — for example, procainamide, hydralazine, anticonvulsants and, less commonly, penicillins, sulfa drugs, and hormonal contraceptives.
Signs and symptoms
The onset of SLE may be acute or insidious and produces no characteristic clinical pattern. However, signs and symptoms commonly include fever, weight loss, malaise, fatigue, rashes, and polyarthralgia. SLE may involve any organ system. (See Signs and symptoms of systemic lupus erythematosus.)
Joint and skin effects
In 90% of patients, joint involvement is similar to that in rheumatoid arthritis (although the arthritis of lupus is usually nonerosive). Most skin lesions are in the form of an erythematous rash in areas exposed to light. The classic butterfly rash over the nose and cheeks occurs in fewer than 50% of the patients. A scaly papular rash (which mimics psoriasis) may also develop, especially in sun-exposed areas. Ultraviolet rays often provoke or aggravate skin eruptions. (See Discoid lupus erythematosus, page 502.) 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.
Cardiopulmonary effects
About 50% of patients with SLE develop signs of pulmonary abnormalities, such as pleurisy, pleural effusions, pneumonitis, pulmonary hypertension and, rarely, pulmonary hemorrhage. Cardiac involvement may include pericardial effusion, pericarditis, myocarditis, endocarditis, and early coronary atherosclerosis.
Renal effects
Onset of glomerulonephritis may be evidenced by microscopic hematuria, pyuria, and urine sediment with cellular casts. Renal disease may progress to kidney failure, particularly when untreated. Urinary tract infections may result from heightened susceptibility to infection.
Neurologic effects
Seizure disorders, peripheral neuropathy, and mental dysfunction may indicate neurologic damage. Central nervous system (CNS) involvement may produce emotional instability, psychosis, and organic brain syndrome. Headaches, irritability, and depression are common.
Systemic effects
Constitutional signs and symptoms of SLE include aching, malaise, fatigue, low-grade or spiking fever, chills, anorexia, and weight loss. Lymph node enlargement (diffuse or local, nontender), abdominal pain, nausea, vomiting, diarrhea, and constipation may occur. Women may experience irregular menstrual periods or amenorrhea during the active phase of SLE.
Because SLE usually strikes women of childbearing age, questions related to pregnancy may arise. Available evidence indicates that a woman with SLE can have a safe, successful pregnancy if she has no serious renal or neurologic impairment.
Diagnosis
Diagnostic tests for patients with SLE include a complete blood cell count with differential, which may show anemia and a decreased white blood cell (WBC) count; platelet count, which may be decreased; erythrocyte sedimentation rate, which may be elevated; and serum electrophoresis, which may show hypergammaglobulinemia.
Specific tests for SLE include the following:
❑ ANA, anti-double-stranded deoxyribonucleic acid (dsDNA), and lupus erythematosus cell tests are positive in patients with active SLE; because the anti-dsDNA test is rarely positive in those with other conditions, it’s the most specific test for SLE. However, if the patient is in remission, anti-dsDNA results may be reduced or absent (because anti-dsDNA correlates with disease activity, especially renal involvement, and helps monitor the patient’s response to therapy). Other autoantibodies commonly found include anti-SM (also highly specific for SLE), anti-SSA, anti-SSB, and anti-RNP.
❑ Urine studies may show red blood cells and WBCs, urine casts and sediment, and significant protein loss (more than 0.5 g/24 hours).
❑ Blood studies reveal decreased serum complement (C3 and C4) levels, which indicate active disease.
❑ Chest X-ray may show pleurisy or lupus pneumonitis.
❑ Electrocardiography may show a conduction defect with cardiac involvement or pericarditis.
❑ Kidney biopsy determines the stage of the disease and the 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, abortion, and thrombocytopenia).
UNDER STUDY: Researchers have noted a significant association between the presence of activated protein C resistance and thrombosis in patients with SLE.
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, such as hydrocortisone or triamcinolone, are recommended for acute lesions.
Refractory skin lesions are treated with an intralesional corticosteroid or antimalarial such as hydroxychloroquine. Because hydroxychloroquine can cause retinal damage, such treatment requires ophthalmologic examination every 6 months.
Corticosteroids
The treatment of choice, cortico-steroids are used 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 usually bring noticeable improvement within 48 hours.
As soon as symptoms are under control, steroid dosage is slowly tapered. (Rising serum complement levels and decreasing anti-dsDNA titers indicate that the patient is responding to treatment.) Diffuse proliferative glomerulonephritis, a major complication of SLE, requires treatment with large doses of steroids and cytotoxic therapy (such as cyclophosphamide). If renal failure occurs, dialysis or a kidney transplant may be necessary. Antihypertensives and dietary changes may also be warranted in patients with renal disease. Patients with SLE who are on long-term steroid therapy are at particular risk for osteonecrosis of the hips.
UNDER STUDY: i>Researchers are studying various biologic agents and immunosuppressants for the treatment of lupus:
❑ Dehydroepiandrosterone is a mild androgen effective in treating mild to moderate lupus; androgens are abnormally low in individuals with lupus.
❑ LJP 394 lowers levels of antibodies to double-stranded DNA and is being used in patients with kidney flare-ups.
❑ B lymphocyte stimulator, an antibody to CD40 ligand, and rituximab are immunotherapy agents.
❑ LJP 1082 is under development for individuals with antibody-mediated thrombosis.
❑ Bromocriptine suppresses prolactin secretion, which is thought to be involved in the onset and progression of lupus.
❑ Thalidomide produces improvement in types of lupus affecting the skin.
Processes, such as apheresis, plasmapheresis, or hematopoietic stem cell transplantation may be used to clean the immune system.
Special considerations
❑ Careful assessment, supportive measures, emotional support, and patient teaching are all important parts of caring for the patient with SLE.
❑ Watch for constitutional signs and symptoms, including joint pain or stiffness, weakness, fever, fatigue, and chills. Observe the patient for dyspnea, chest pain, and any edema of the extremities.
❑ Check urine for hematuria, scalp for hair loss, and skin and mucous membranes for petechiae, bleeding, ulceration, pallor, and bruising.
❑ Provide a balanced diet. Foods high in protein, vitamins, and iron help the patient maintain optimum nutrition and prevent anemia. However, renal involvement may mandate a lowsodium, 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 her 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, and watch for adverse reactions, especially when the patient is taking high doses of a corticosteroid.
❑ Advise the patient receiving cyclophosphamide to drink plenty of fluids.
❑ Monitor the patient’s vital signs, intake and output, weight, and laboratory reports. Check pulse rates, and observe her for orthopnea. Check stools and GI secretions for blood.
❑ Observe the patient for hypertension, weight gain, and other signs of renal involvement.
❑ Assess the patient 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.
❑ Refer the patient to the Lupus Foundation of America and the Arthritis Foundation as necessary.
❑ For more patient-teaching information, see Managing lupus.
Pictures
Book Source Details
- Book Title: Handbook of Diseases
- Author(s): Springhouse
- Year of Publication: 2003
- Copyright Details: Handbook of Diseases, Copyright © 2003 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: Handbook of Diseases
Authors: Springhouse
Publisher: Lippincott Williams & Wilkins
Copyright: 2003
ISBN: 1-58255-266-5
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