Arthritis affects approximately one child in every thousand in a given year. Fortunately most of these cases are mild. However, approximately one child in every 10,000 will have more severe arthritis that doesn't just go away.
Juvenile Rheumatoid Arthritis (JRA)
Juvenile rheumatoid arthritis (JRA) is the most common type of arthritis that persists for months or years at a time. There are three main forms of JRA - pauciarticular, polyarticular, and systemic onset - that are separated by how they begin.
Pauciarticular JRA involves less than four joints at the beginning. This often begins in young girls as a swollen knee or ankle that appears without injury or explanation. Usually it is painless, but someone notices that the knee looks swollen or the child is walking funny. Since arthritis causes morning stiffness, parents may be slow to get concerned 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: eye inflammation and uneven leg length.
The biggest problem is that many children with pauciarticular 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 this eye disease is more common in children with a positive test for antinuclear antibodies (ANA), such children must be examined by an eye specialist every three month. All other children with 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 it's important to make sure every child's eyes get checked.
The second important problem with pauciarticular JRA is that it may cause the bones in the legs to grow at different rates. Thus, one leg becomes longer than the other. When this happens, children 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. Such knee and hip damage can and should be prevented.
Fortunately, we understand how this happens. When the knee or another joint is inflamed by arthritis, its blood supply increases. Then, just like a plant that receives more water than other plants, it grows faster and larger. Of course, we try stop the inflammation. Most often the therapy is successful and the child does not develop a significant leg length discrepancy. If it does happen, two steps can be taken.
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 orthopaedist should look at x-rays of the legs and try to forecast when the bones are going to stop growing. If the leg with arthritis is 3 cm longer than the other leg, based on a review of the x-rays, they try to forecast when there is 3 cm of leg growth left. Then, with a very simple operation, they can stop the growth on the leg that is too long, allowing the short leg to catch up.
Polyarticular JRA is the form in which four or more joints are involved from the beginning. This form is more severe because of the greater number of joints involved and because 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. It is critically important to bring the disease under control as quickly as possible in order to help prevent permanent joint damage. This may require use of some fairly strong medications.
In severe cases, corticosteroid medications (such as prednisone) may be necessary, but they are not a real solution. Steroids make patients with arthritis feel wonderful, but it's like sweeping dirt under the rug. Everything looks good, but it really isn't. Taking too much steroid for long periods causes lots of problems, such as short stature and weak bones. Whenever we have to put children on steroids, we want to get them off as quickly as possible.
Nonsteroidal antiinflammatory drugs (NSAIDs) are enough for many children with polyarticular JRA, but more severe cases may require gold shots, or second line immunosuppressant medications, such as sulfasalazine or methotrexate.
Systemic onset JRA is the most worrisome form of JRA. It begins with high fevers and a rash. It is critically important to make sure the patient really has systemic onset JRA and not an infection of some kind. One of the most important indicators of systemic onset JRA is that the fever goes away for at least part of every day. Usually, the fever is high once or twice each day. At those times, children look very sick and don'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. In some patients, it goes completely away and never comes back again. In others, the fevers and rash go away, but the arthritis progresses over time and can be very severe. This form of JRA can involve the internal organs and, rarely, can be 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.
Other Forms of Arthritis in Children and Adolescents
There are other forms of arthritis that can affect children and adolescents, and some doctors lump them together with JRA. But they have different outcomes and should be considered separately.
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.
Many types of arthritis are more likely to affect older children (those older than eight years of age) and teenagers, while typical JRA most often affects young children.
One example 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 that 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 that is starting early. Because it is starting so early, this is also very worrisome group, and these children need to be treated aggressively. Often they will have lifelong arthritis.
A second form of arthritis 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 JRA because the best treatment is different, and the outcome is likely to be different. In addition, one must look carefully for evidence of 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 boys who are positive for a test for a genetic marker called HLA B27. 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, physicians have only recognized children with spondyloarthropathies as being 'different' since the middle 1970s; so good long-term follow-up data is not available yet.
A number of other rheumatic diseases also can occur in childhood. These include systemic lupus erythematosus, Lyme disease, Kawasaki disease (marked by symptoms which typically include a fever that lasts for at least 5 days, red eyes, a body rash, and severely-chapped lips and mouth), scleroderma, dermatomyositis, and Raynaud's phenomenon (painful hands or feet in response to cold).
The Future for Youngsters with Arthritis
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 wheelchair. Every doctor knows stories of children who looked awful, but did very well or looked like it was nothing serious but became very sick, but these are the rare exceptions. We can take good care of children with arthritis. For more than 95% of the children with arthritis, we don't need new drugs or miraculous inventions, we just need proper application of the resources we already have.
There are three important issues for every child or adolescent with arthritis:
- proper recognition and diagnosis of the disease;
- proper treatment by an experienced physician with a multidisciplinary support including physical and occupational therapists and orthopaedic surgeons;
- proper education of the patient and family.
Further, children with arthritis must be treated just like everyone else. They need the same discipline, the same allowance, the same grades, and the same respect as all the other children. Arthritis might affect the body, but it must never be allowed to affect the mind.
People with arthritis are no different from everyone else in the world. They all need to grow up, have jobs, and develop interpersonal relationships. Some will have some difficulty with mechanical problems. Many will have small things they can't do if you watch them carefully. Few will go on to be professional athletes but, if treated properly, even fewer will be totally disabled by their disease.
Far more children have been disabled because they were told they couldn't do things than those who were really disabled by their disease. Parents should never accept being told that their child is condemned to a wheelchair. In almost every case, we should be able to correct the problem and get them walking again.
That is why getting educated about your child's disease and obtaining quality medical care is so very important.
When to Seek Referral to a Specialist
The care of a child with arthritis should involve a coordinated treatment program by a multidisciplinary team, including the primary care physician (usually the pediatrician), the pediatric rheumatologist, the physical therapist, and other members of the health care system.
Parents should consider consultation with a pediatric rheumatologist in the following situations:
- To confirm the diagnosis - or to obtain a diagnosis in a child with any of the symptoms described above;
- At the initiation of therapy, in order to develop the best medication and physical therapy regimen;
- Whenever corticosteroids or immunosuppressant drugs are recommended;
- During the course of the illness, to assess the child's response to therapy and, if necessary, to change the treatment regimen;
- If possible medication side effects are causing problems;
- If the disease does not improve or worsens despite therapy;
- If any functional limitation develops;
- Prior to consideration of any surgery.
