1. Malar rash: a red rash over the cheeks (often crossing over the nose)
2. Discoid rash: a scaly red rash (uncommon in children)
3. Photosensitivity: burns easily in sun, rashes and sensitivity to light
4. Mucosal ulcers: sores in the mouth or nose
5. Serositis: inflammation of the lining of the chest or abdomen, producing pain
6. Arthritis: pain swelling or limitation of motion of a joint
7. Renal involvement: abnormalities on the urine tests or blood tests
8. Neurologic involvement: problems with seizures or difficulty thinking normally
9. Hematologic involvement: abnormalities of the red cells, white cells, or platelets
10. Immunologic disorder: abnormal blood tests characteristic of SLE
11. Antinuclear antibodies
A child is considered to have definite SLE if he or she has any four of the eleven criteria.
Although the onset of SLE typically occurs between the ages of twelve and twenty-five years in most patients, it can occur in children as young as three. The incidence of SLE varies by sex and race, but from my experience I find that among girls between nine and nineteen it occurs most frequently in Asians and next most frequently in African Americans, followed by Hispanics and whites. The number of affected boys is much, much smaller. Relatives of someone with SLE are at greater risk of developing SLE than the rest of the population.
Although chronic failure to thrive is the most common presentation in children, SLE can also begin in many other ways. Chronic swelling of the hands and feet, easy bruising, joint pains, abnormal urine tests, photosensitivity, seizures, altered personality and depression, and even altered school performance all may be initial findings. However, it is important to remember that these are nonspecific symptoms that may occur in many different illnesses.
Some children with SLE reach my office after a long history of being evaluated for problems such as anemia, fatigue, fever, and weight loss without explanation. Less often the onset of SLE is sudden and dramatic, with bleeding from the lungs, kidney problems, or multiple organ involvement. Again, it is important to remember that SLE is not the only disease that may cause these findings.
Once the diagnosis of SLE is suspected, proper evaluation begins with testing for ANA. As noted, if the ANA test is negative, it is extremely unlikely that the child has SLE. If the test for ANA is positive, further evaluation should include a complete blood count (CBC), chemistry panel, clotting studies, urinanalysis, and testing for Ro, La, Sm, RNP, and anti-DNA antibodies (see Chapter 22). Measurements of complement levels C3 and C4 should be performed. It is also useful to screen these children for thyroid function abnormalities, antithyroid antibodies, anticardiolipin antibodies, and rheumatoid factor. Sometimes after these tests it will be clear that a child has SLE. In other cases, there are several abnormalities but not enough to make a definite diagnosis of SLE.
The diagnosis of definite SLE requires that the child fulfill four of the eleven American College of Rheumatology criteria for the diagnosis of definite lupus. However, there are many children with SLE who initially fulfill fewer than four criteria. If children have several findings that suggest SLE but do not fulfill criteria for a definite diagnosis of SLE, they should be monitored carefully. It really does not matter whether they are said to have SLE, possible SLE, or probable SLE. The key to proper care for these children is to treat appropriately whatever problems they are having.
Children who have nonspecific symptoms and a positive ANA require routine follow-up to look for evidence of disease. Some physicians simply instruct the parents to bring the child back if more problems develop. I prefer to be proactive and periodically look for problems. This often allows me to begin therapy before the problems become more serious. It is well known that children with SLE may develop kidney disease and damage to other organs without initially complaining of pain or discomfort. It is important to detect these problems as early as possible and to minimize the damage to vital organs.
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