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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:

• malar or discoid rash
• photosensitivity
• oral or nasopharyngeal ulcerations
• nonerosive arthritis (of two or more peripheral joints)
• pleuritis or pericarditis
• profuse proteinuria (exceeding 0.5 g/day) or excessive cellular casts in the urine
• seizures or psychoses
• hemolytic anemia, leukopenia, lymphopenia, or thrombocytopenia
• anti-dsDNA or antiSmith antibody test or positive findings of antiphos­pholipid antibodies (elevated IgG or IgM anticardiolipin antibodies, positive test for lupus anticoagulant, or false­positive serologic tests for syphilis)
• abnormal titer of antinuclear anti­body.

Diagnosis

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:

• ANA, anti-double-stranded deoxyribonucleic acid (dsDNA), and lupus erythematosus cell tests are positive in active SLE; because the anti-dsDNA test is rarely positive in other conditions, it's the most specific test for SLE. However, if the patient is in remission, antidsDNA 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 auto antibodies 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 white blood cells, 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).

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

• Careful assessment, supportive measures, emotional support, and patient teaching are all important parts of caring 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. Foods high in protein, vitamins, and iron help maintain optimum nutrition and prevent anemia. However, 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 his progress.
• 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.
• Avoid intense sun exposure because it can trigger symptoms. Wear a wide­brimmed hat and long-sleeved clothing outdoors, and stay indoors during the brightest hours of the day. Always apply sunblock and repeat the application to attain the best protection.

Related information

• Discoid lupus erythematosus