What is this Condition?
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, which affects multiple organ systems (as well as the skin) and can be fatal.
Like rheumatoid arthritis, systemic lupus is characterized by recurring remissions and flare-ups, especially common during the spring and summer. The annual incidence of this disorder averages about 27 cases in 1 million whites and 75 cases in 1 million blacks. Systemic lupus strikes women 8 times as often as men, increasing to 15 times as often during childbearing years. Systemic lupus occurs worldwide but is most prevalent among Asians and blacks.
The prognosis improves with early diagnosis and treatment but remains poor for people who develop heart and blood vessel, kidney; or nervous system complications or severe bacterial infections.
What Causes it?
The exact cause of systemic lupus remains a mystery, but available evidence points to interrelated immunologic, environmental, hormonal, and genetic factors. An immune dysfunction, such as autoimmunity, is considered the prime causative mechanism. In autoimmunity, the body produces antibodies (such as the antinuclear antibody) against its own cells. The formed antigen-antibody complexes can suppress the body’s normal immunity and damage tissues. One significant feature in people with this disorder 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 .
Certain predisposing factors may make a person susceptible to systemic lupus. Physical or mental stress, streptococcal or viral infections, exposure to sunlight or ultraviolet light, immunization, pregnancy, and abnormal estrogen metabolism all may affect the disease’s development.
Systemic lupus also may be triggered or aggravated by treatment with certain drugs - for example, Procan SR, Apresoline, antiseizure drugs and, less frequently; penicillins, sulfa drugs, and oral contraceptives.
What are its Symptoms?
The onset of systemic lupus 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 pain in multiple joints.
Systemic lupus may involve every organ system. In 90% of cases, joint involvement is similar to that in rheumatoid arthritis. Skin lesions are most commonly a red rash in areas exposed to light. The classic butterfly rash over the nose and cheeks occurs in fewer than 50% of people with systemic lupus. Ultraviolet rays often provoke or aggravate skin eruptions. Vasculitis can develop (especially in the fingers and toes), possibly leading to infarctive lesions, necrotic leg ulcers, or gangrene of the fingers or toes. Raynaud’s phenomenon (decreased blood flow to the fingers and toes, causing numbness and pain) appears in about 20% of cases. Patchy hair loss and painless ulcers of the mucous membranes are common.
Constitutional symptoms of systemic lupus include aching, malaise, fatigue, low-grade or spiking fever, chills, loss of appetite, and weight loss. Lymph node enlargement (diffuse or local, and nontender), abdominal pain, nausea, vomiting, diarrhea, and constipation may occur. Women may experience absent or irregular menstrual periods during the active phase of systemic lupus erythematosus.
About 50% of people with systemic lupus develop signs of cardiopulmonary abnormalities, such as pleuritis, pericarditis, and shortness of breath. Myocarditis, endocarditis, rapid heartbeat, parenchymal infiltrates, and pneumonitis may occur. Kidney effects may include blood, protein, sediment, and cellular casts in the urine and may progress to total kidney failure. Urinary tract infections may be caused by heightened susceptibility to infection. Seizure disorders and mental dysfunction may indicate neurologic damage. Central nervous system involvement may produce emotional instability, psychosis, and organic brain syndrome. Headaches, irritability, and depression also are common.
How is it Diagnosed?
Diagnostic tests for people with systemic lupus include a complete blood count with differential, platelet count, erythrocyte sedimentation rate, and serum electrophoresis.
Specific tests include antinuclear antibody, anti-DNA, and lupus erythematosus cell tests, urine studies, blood complement studies, chest X-ray, electrocardiography, and kidney biopsy.
Some people show a positive lupus anticoagulant test and a positive anticardiolipin test. These people are prone to antiphospholipid syndrome (blood clot formation, miscarriage, and low platelet count).
How is it Treated?
People with mild systemic lupus require little or no medication. Nonsteroidal anti-inflammatory compounds, including aspirin, control arthritis symptoms in many cases. Skin lesions need topical treatment. Corticosteroid creams, such as Cordran, are recommended for acute lesions.
Refractory skin lesions are treated with intralesional corticosteÂroids or antimalarials, such as Plaquenil and Ara1en. Because these drugs can cause retinal damage, such treatment requires eye exams every 6 months.
Corticosteroids remain the treatment of choice for systemic symptoms of systemic lupus, for acute generalized flare-ups, or for serious disease related to vital organ systems, such as pleuritis, pericarditis. lupus nephritis, vasculitis, and central nervous system involvement. Initial doses equivalent to 60 milligrams or more of Orasone often bring noticeable improvement within 48 hours. As soon as symptoms are under control, corticosteroid dosage is tapered down slowly.
Diffuse proliferative glomerulonephritis, a major complication of this disorder, requires treatment with large doses of corticosteroids. If kidney failure occurs, dialysis or kidney transplant may be necessary. In some people, cytotoxic drugs such as Imuran and Cytoxan may delay or prevent deteriorating kidney status. Antihypertensive drugs and dietary changes may also be warranted in kidney disease.
The light-sensitive person should wear protective clothing (hat, sunglasses, long sleeves, slacks) and use a sunscreen containing para-aminobenzoic acid when outside. Because systemic lupus usually strikes women of childbearing age, questions associated with pregnancy often arise. The best evidence available indicates that a woman with this disorder can have a safe, successful pregnancy if she has no serious kidney or nervous system impairment.
What can a person with lupus do?
• Watch for symptoms, such as joint pain or stiffness, weakness, fever, fatigue, and chills. Stay alert for shortness of breath, chest pain, and arm or leg swelling. Check for blood in your urine, scalp hair loss, and bleeding, ulcers, pallor, and bruised skin and mucous membranes.
• Eat a balanced diet. Foods high in protein, vitamins, and iron help maintain optimum nutrition and prevent anemia. However, if you have kidney problems, the doctor will recommend a low-sodium, low-protein diet.
• Get plenty of rest.
• Apply heat packs to relieve joint pain and stiffness. Get regular exercise to maintain a full range of motion and prevent contractures. Perform range-of-motion exercises, as well as body alignment and postural techniques.
• Take prescribed drugs exactly as directed, and watch for side effects, especially if you’re taking high doses of corticosteroids.
• If you’re receiving Cytoxan, be sure to drink plenty of fluids.
• Use hypoallergenic makeup, if needed, and consult a hairdresser who specializes in scalp disorders.
• Buy drugs in quantity, if possible. Be skeptical about “miracle’ drugs for relief of arthritis symptoms.
• Contact the Lupus Foundation of America and the Arthritis Foundation for more information.