Lupus, Part 1 — Introduction, Incidence and Symptoms

What is systemic lupus erythematosus?

Systemic lupus erythematosus, aka SLE or lupus (LOO'-pus), is an episodic, multisystem disease characterized by widespread inflammation of the blood vessels and connective tissues. Lupus is a chronic disease, but symptoms may come and go after the initial diagnosis. Lupus is unpredictable and dangerous. Management requires regular care from doctors who are skilled in treating children. The cause is unknown, but sun exposure can trigger lupus in susceptible individuals. Certain medications have side effects that mimic lupus (drug-induced lupus), which usually resolve once the medication is stopped.

Incidence and prevalence

The incidence of lupus varies by location and ethnicity. In the U.S. population, rates of lupus among children younger than 15 have been reported between 0.5 and 0.6 cases per 100,000. The diagnosis is more common among females, and the incidence among girls seems to spike during puberty. Prior to puberty, the female-to-male ratio is approximately 2-to-1. Following puberty, the ratio is 4-to-1. The prevalence of lupus varies widely, ranging from 4 cases to 250 cases per 100,000. It is more prevalent among Native American, Asian, Latin American and African American patients. In one study of adults, the prevalence of lupus among black women was approximately 1 per 500.

The five-year survival rate for children with lupus is more than 90 percent. Most deaths in children with lupus are due to infection, renal failure, pulmonary hemorrhage, or involvement of the central nervous system (brain and spinal cord). A heart attack (myocardial infarction) may occur in young adulthood as a complication of long-term corticosteroid use, and may be seen years after its use.

Symptoms

Approximately 20 percent of patients with lupus are diagnosed by age 10. Although the onset of lupus has been reported in the first year of life, lupus remains uncommon before age 8.

At the time of diagnosis, the most common symptoms of lupus are fever lasting several days, general weakness, and evidence of multisystem involvement. A child might have fatigue, joint pain, rash and fever. Some children will show other symptoms, such as memory loss, psychosis, transverse myelitis (swelling of the spinal cord), hemoptysis (coughing up blood), swelling of the legs, headache, and painful mouth sores. Many children develop a rash on the face or chest, particularly after being in the sun.

Certain portions of this overview of lupus will likely be of interest only to physicians and other professionals.

Eleven criteria are used for the classification of lupus in adults. The same criteria can serve as a guideline in children. Any four criteria are sufficient for diagnosis, and these should be sought in the history. Of note, the anti-nuclear antibody (ANA) test is almost always positive. Diagnosis is not difficult in the child who presents with many manifestations, such as malar rash, arthritis, chest pain, abnormal urine, and a positive ANA. Some patients present symptoms over longer periods and require careful consideration. Occasionally, a definite diagnosis never develops or the patient may have an overlap syndrome with characteristics of several rheumatic diseases. Joints may become swollen and stiff. Mouth ulcers may come and go. Sometimes more hair falls out than the normal amount. The hands may turn red, white and blue when exposed to cold, such as gripping a cold drink or going outside in the cold weather. Children may also present with internal organ involvement in the kidneys, brain, heart and lungs.

Laboratory tests can help diagnose lupus and determine whether any internal organs are involved. Regular testing of blood and urine is useful to monitor the activity and severity of the disease, the effectiveness of medications, and how well the medications are tolerated. Typical laboratory tests include anti-nuclear antibody (ANA), anti-DNA, other antibody tests, complete blood count (CBC), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement levels, urine tests, and other specific tests to monitor involvement of internal organs.

Children with inflammation of the kidneys may develop chronic renal disease; those with brain involvement may have seizures, serious mood changes or hallucinations; others may have fluid around the heart and lungs; some develop painful arthritis.


For more information

Part 2 — Treatment, Side-Effects, Restrictions and Implications for School

Lupus Foundation of America, Inc.

www.lupus.org
2000 L Street NW, Suite 710
Washington, DC 20036

Phone 202-349-1155
Fax 202-349-1156

Arthritis Foundation

http://www.arthritis.org/
P.O. Box 7669
Atlanta, GA 30357-0669

Phone 800-283-7800
Information about lupus in children and adolescents.

Contributed by:

Carol Lindsley, MD
Professor
Division Chief, Pediatric Rheumatology
Department of Pediatrics
University of Kansas Medical Center

Kathy Davis, MSEd, PhD
Associate Professor
Project Director, Connected Kansas Kids
Director, KU Kids Healing Place
University of Kansas Medical Center