Arthritis In Childhood - Juvenile Arthritis, Juvenile Rheumatoid Arthritis

For both children with arthritis and the doctors who care for them public education is one of the greatest challenges. Most lay people and many doctors fail to realize that the problem exists. Many children suffer for months or years before the diagnosis of arthritis is thought of and proper treatment begun. But the problem doesn’t end there. Children with arthritis frequently experience difficulty because their teachers and schoolmates don’t believe children can get arthritis and have no idea what to expect from the child with arthritis or about the nature of the illness. As a result, when the child is finally diagnosed with arthritis the family may be told just to put them in a wheel chair because ’nothing can be done.’ This is entirely wrong!!



Arthritis affects approximately 1 child in every thousand in a given year. Fortunately most of these cases are mild. However, approximately one child in every ten thousand will have more severe arthritis that doesn’t just go away. Many children have what is called an acute reactive arthritis following a viral or bacterial infection. This arthritis is often quite severe for a brief period, but usually disappears within a few weeks or months. Juvenile rheumatoid arthritis (JRA) is the most common type of arthritis which persists for months or years at a time.





There are three main forms of juvenile rheumatoid arthritis (JRA) which are separated by how they begin. Pauciarticular juvenile rheumatoid arthritis (JRA) is defined by the involvement of less than four joints at the beginning. This is the form which often begins in young girls as a swollen knee or ankle which appears without injury or explanation. Usually it is ’painless’ but someone will have noticed that the knee looks swollen or the child is walking funny. Since arthritis causes morning stiffness parents are slow to get concerned about this because, ’She always looks okay once she gets going.’ This arthritis is often very mild and treated just with mild nonsteroidal anti-inflammatory drugs, but it can cause two important problems. The biggest problem is that many children with pauciarticular juvenile rheumatoid arthritis (JRA) develop inflammation of the eye (iridocyclitis). The inflammation is not painful, but if not detected and treated it may lead to scarring of the lens and permanent visual damage (even blindness). At the beginning this inflammation cannot be seen except by an ophthalmologist using a special instrument called a ’slit lamp.’ Because the eye disease is more common in children with a positive test for antinuclear antibodies (ANA) these children are all told they need every three month examinations by an eye specialist. All other children with juvenile rheumatoid arthritis (JRA) need eye examinations every six months. No one has been able to completely explain the association of eye disease and arthritis or why it is more frequent in children with ANA. But we do know it happens and its important to make sure every child’s eyes get checked.

The second important problem with pauciarticular juvenile rheumatoid arthritis (JRA) is that it may cause the bones in the legs to grow at different rates with the result that one leg is longer than the other. When this happens children are forced to walk with a limp. This damages the knee and the hip leading to premature arthritis from ’wearing out’ the joints by the time the child is an adult and should be prevented. Fortunately we understand how this happens. When the knee or another joint is inflamed by the arthritis its blood supply increases. Then just like a plant that receives more water than the plants around it, it grows faster and larger. We are always trying to stop the inflammation. Most often the therapy is successful and the child does not develop a significant leg length discrepancy. If he or she does we can do two things. First we can put a lift in the shoe on the short side to correct the effect of the different leg lengths. This doesn’t do anything for the knee, but it prevents excessive wear on the hip and allows the child to walk more normally. The next step is to monitor growth. When the child is getting closer to fully grown an orthopedist can look at X-rays of the legs and try to guess when the bones are going to stop growing. If the leg with arthritis is 3 cm longer than the other leg, they will look at the X-rays and try to guess when there is 3 cm of leg growth left. Then you stop the growth on the leg that is too long and allow the short leg to catch up. This can be done with a very simple operation.



Polyarticular juvenile rheumatoid arthritis is the form in which four or more joints are involved from the beginning. This form is more severe both because of the greater number of joints involved and the fact that it tends to get worse over time. These children may have a great deal of difficulty with normal activities and need to be treated aggressively.

From a doctor’s point of view, the most important thing is to bring the disease under control as quickly as possible. Typically, this involves at least medications that reduce inflammation, nonsteroidal antiinflammatory drugs (NSAIDs). This may also require use of some fairly strong medications, but it’s important to recognize that they are necessary to reduce symptoms and prevent permanent damage. One thing to watch out for is using steroids (e.g. prednisone). In severe cases this may be necessary, but it is not a ’real’ solution. Steroids make patients with arthritis feel wonderful, but its like sweeping dirt under the rug. Everything looks good, but it really isn’t. Taking too much steroid for a long period causes lots of problems like short stature and weak bones. Whenever we are required to put a child on steroid medications we want to wean them as quickly as possible. Nonsteroidal anti-inflammatory drugs are enough for many children with polyarticular juvenile rheumatoid arthritis, but more severe cases may require more aggressive "second line" medications, such as gold shots, sulfasalazine or methotrexate. Severe cases requiring steroids or "second line" medications should be under the care of experienced physicians.

A new form of medications, called TNF-blockers is now available. TNF-alpha is a substance made by cells of the body that has an important role in promoting inflammation. By blocking the action of TNF-alpha, TNF-blockers reduce the signs and symptoms of inflammation. Etanercept (Enbrel) is a TNF-blocker that is injected into the skin twice weekly and is indicated for reduction in signs and symptoms of moderately to severely active polyarticular-course juvenile rheumatoid arthritis in patients who have had an inadequate response to one or more disease-modifying medicine(s). Remicade (infliximab) is an intravenously infused antibody that blocks the effects of tumor necrosis factor alpha (TNF-alpha). Remicade, an approved treatment for adult rheumatoid arthritis, is given by intravenous infusion every 2 months. Remicade was recently reported as effective for treating juvenile rheumatoid arthritis, thereby resulting in a significant and prompt reduction in disease activity and improved quality of life. Another scientific paper presented data recently demonstrating that doses of Remicade that are higher than currently recommended doses could be effective and necessary in juvenile rheumatoid arthritis.



The most worrisome form of juvenile rheumatoid arthritis is systemic onset 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 an infection of some kind. 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. This form of arthritis is puzzling to physicians. 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. In addition to their other problems these children have an greater likelihood of bad reactions to medications and must be monitored very carefully.



There are several other forms of arthritis which can affect children and adolescents which some doctors lump together with juvenile rheumatoid arthritis, but have different outcomes and should be considered separately. Interestingly these most often affect older children (greater than eight 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.

A second form of arthritis which 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 (i.e. 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 around their tendons. 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 best treatment is different and the 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, Reiter’s syndrome, 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 1970’s so good long term follow-up data is available yet.



With proper therapy the children with all of these forms of arthritis will usually get better over time. Indeed, the vast majority of children with arthritis grow up to lead normal lives without significant difficulty. Even for severe cases with proper medications, proper physical and occupational therapy, and proper surgery if necessary, virtually no one with arthritis should need a wheel chair. Everyone’s doctor knows stories of children who looked awful, but did very well or looked like it was ’nothing serious,’ who became very sick, but these are the rare exceptions. We can take good care of children with arthritis. For over 95% of the children with arthritis today we don’t need new drugs or miraculous inventions, we just need proper application of the resources we already have.

There are three important things for every ch