Juvenile Arthritis (cont.)
Systemic-onset juvenile rheumatoid arthritis
Often the most difficult form of juvenile rheumatoid arthritis is systemic-onset JRA, also known as Still's disease. This form of juvenile rheumatoid arthritis begins with high fevers and a rash. It is very important in this setting to make sure the patient really has systemic-onset juvenile rheumatoid arthritis and not another condition, such as infection. One of the most important findings is that the fever goes away for at least part of every day in someone with systemic-onset juvenile rheumatoid arthritis. Usually the fever is high once or twice each day. At those times, the child looks very sick and doesn't want to be touched, but when the fever goes down to normal again, they look and feel better. Sometimes it goes completely away and never comes back again. Other times, the fevers and rash go away, but the arthritis progresses over time and can be very severe. This form of juvenile rheumatoid arthritis can involve the internal organs and rarely is a
"life-threatening" disease.
Treatments for systemic-onset JRA include nonsteroidal antiinflammatory drugs (NSAIDs such as ibuprofen and naproxen), hydroxychloroquine (Plaquenil), cortisone medications (such as prednisone and prednisolone), methotrexate, and for resistant disease, anakinra (Kineret). Some research has suggested that thalidomide may be an effective treatment for children with systemic-onset JRA.
What are some other forms of arthritis which can affect children?
There are several other forms of arthritis that can affect children and adolescents that can be considered separately from juvenile rheumatoid arthritis. Interestingly,
these most often affect older children (greater than 8 years of
age) and teenagers, while typical juvenile rheumatoid arthritis most often affects young
children. One of these is the teenager who has rheumatoid factor-positive arthritis with involvement of the small joints in the hands
and feet. Rheumatoid factor is a blood-test finding which is present
in most adults with rheumatoid arthritis but is absent in most
children with JRA. It is present in this group because they usually
are teenagers who have adult-type rheumatoid arthritis starting
early. Because it is starting early, this is a very worrisome group,
and these children need to be treated aggressively. Often they will
have lifelong arthritis.
Another form of arthritis that is common in this "older" group is spondyloarthropathy. This is a family of diseases in which the arthritis is the same, but the associated problems are very different. The typical findings of a spondyloarthropathy are early involvement of the hips and other large joints. In addition, these forms of arthritis tend to be asymmetric (one side of the body is more severely affected than the other). The key finding is that these children not only have inflamed joints, but they also have inflammation at the points where tissues attach to bone, such as tendons and ligaments. Often they have ankle or heel pain due to inflammation of the tendons inserting in the foot. In some mild cases, the tendon inflammation occurs without obvious swollen joints.
It is important to recognize the spondyloarthropathies as different from juvenile rheumatoid arthritis because the optimal treatment, monitoring, and outcome is likely to be different. In addition, one must look carefully for evidence of the other diseases that can be associated with spondyloarthropathies. These include inflammatory bowel disease, psoriasis, reactive arthritis, and Behcet's syndrome. The most worrisome children with spondyloarthropathies are the HLA B27-positive boys. They are at risk for developing ankylosing spondylitis. However, most children with spondyloarthropathies seem
to do reasonably well. In general, for children who are HLA B27
negative and do not have an associated condition, the arthritis is
more likely than JRA to come and go repeatedly over a period of
years but is less likely to be very severe or destructive.
Unfortunately, we have only recognized children with spondyloarthropathies as being
"different" since the middle 1970s, so
no good long-term follow-up data is available yet.
Sometimes, children with a form of chronic skin inflammation, called psoriasis, can develop arthritis. This form of arthritis is referred to as psoriatic arthritis. Occasionally, there is a family history of psoriasis that can help to clue the doctor into this diagnosis.
Next: What is the outlook (prognosis) for children with arthritis? »
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