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Juvenile Lupus (SLE)

Juvenile lupus (also known as juvenile systemic lupus erythematosus or juvenile SLE) is an autoimmune disease in which the body produces antibodies which attack its own cells. This causes inflammation and subsequent damage to multiple organs, including the skin, joints, kidneys, nervous system, heart and lungs. Though the exact cause of lupus is unknown, a combination of genetic predisposition and environmental factors is thought to be the source. Lupus and other autoimmune diseases often run in families, and in susceptible people, sunlight may trigger the disease. Though lupus is typically diagnosed in adulthood, teenagers and (rarely) younger children can develop lupus as well. The disease is more common in females, although males can also be affected. Some patients who have only some of the signs and symptoms of lupus may actually have mixed connective tissue disease or overlap syndrome instead instead.

Symptoms

Lupus can affect each person differently, and the disease can range from very mild to life threatening. Some people are very sick at initial presentation, while in others, lupus may take years to cause significant symptoms. Rashes, especially on the face, are common in people with lupus, as are systemic symptoms like fever, weight loss, loss of appetite and fatigue. Other symptoms include arthritis, hair loss, sores in the mouth or nose, and swollen lymph nodes. People with lupus may also have problems with their kidneys, heart, lungs, blood or brain.

Diagnosis

Because lupus is rare in children, it may take weeks or even months of symptoms before it is diagnosed. A pediatric rheumatologist is the best person to diagnose lupus in a child. There is no one specific test for lupus; rather, the diagnosis is made based on a combination of the patient’s symptoms, a physical exam by a doctor, and laboratory findings. Most people with lupus test positive for certain autoantibodies, which are antibodies the body makes against its own cells. Some people may have these autoantibodies without actually having lupus or another disease. Depending on the patient’s symptoms and test results, the physician may order additional tests, including studies of the heart, lungs, kidneys or brain.

Treatment

There is no cure for lupus, but medications that suppress the immune system can effectively treat the disease. The precise treatment for each person lupus depends on how severe the disease is and which organs are affected. Many patients with lupus require steroid medications at the time of diagnosis. But, because steroids have significant side effects, these medications are typically reduced or eliminated as quickly as can be done safely. Most patients with lupus are treated with a medication called hydroxychloroquine (Plaquenil), which is well-tolerated and improves long-term prognosis. Some patients benefit from oral immunosuppressive drugs, while others require intravenous (IV) medication that is given in a hospital or an outpatient facility. Important nonmedical interventions include rest and stress reduction, a well-balanced diet and regular exercise, the use of sunscreen and avoidance of sun exposure

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