Systemic Lupus Erythematosus - Symptoms And Treatment
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 the spring and summer.
The annual incidence of SLE averages 27.5 cases per 1 million whites and 75.4 cases per 1 million blacks. SLE strikes women 8 times as often as men, increasing to 15 times as often during 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.
Causes of Systemic Lupus Erythematosus
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. In 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 frequently, penicillins, sulfa drugs, and oral contraceptives.
Signs and symptoms
The onset of SLE may be acute or insidious and produces no characteristic clinical pattern. However, symptoms commonly include fever, weight loss, malaise, Kidney problems (protein leak), fatigue, rashes, and polyarthralgia. SLE may involve any organ system.
SIGNS OF SYSTEMIC LUPUS ERYTHEMATOSUS
Diagnosing systemic lupus erythematosus (SLE) is difficult because SLE often mimics other diseases, symptoms may be vague, and symptoms vary greatly from patient to patient.
For these reasons, the American Rheumatism Association has issued a list of criteria for classifying SLE, to be used primarily for consistency in epidemiologic surveys. Usually, four or more of these signs are present at some time during the course of the disease:
Diagnostic tests for patients with SLE include a complete blood count with differential, which may show anemia and a decreased white blood cell count; platelet count, which may be decreased; erythrocyte sedimentation rate, which is often elevated; and serum electrophoresis, which may show hypergammaglobulinemia.
Specific tests for SLE include the following:
Some patients show a positive lupus anticoagulant test and a positive anticardiolipin test. Such patients are prone to antiphospholipid syndrome (thrombosis, abortion, and thrombocytopenia).
Treatment of Systemic Lupus Erythematosus
Patients with mild disease require little or no medication. Nonsteroidal antiinflammatory compounds, 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 intralesional corticosteroids or antimalarials such as hydroxychloroquine. Because hydroxychloroquine can cause retinal damage, such treatment requires ophthalmologic examination every 6 months.
Corticosteroids :- The treatment of choice, corticosteroids 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 often bring noticeable improvement within 48 hours.
As soon as symptoms are under control, steroid dosage is tapered down slowly. (Rising serum complement levels and decreasing anti-dsDNA titers indicate that the patient is responding to the 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. Antihypertensive drugs and dietary changes may also be warranted in renal disease. SLE patients on long-term steroids are at a particular risk for osteonecrosis of the hips.
Special considerations and prevention
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