Juvenile Rheumatoid Arthritis (JRA)

    JRA is also called juvenile chronic arthritis (JCA), juvenile idiopathic arthritis (JIA), or juvenile arthritis (JA).  Why so many names?  Because it is not really one disease.  It is a grouping of many different diseases all of which cause children to have swollen joints.  Initially doctors did not recognize that there were many causes of arthritis in childhood and all of these diseases were lumped together.  Now we understand that there are at lease 8 different types of childhood arthritis and perhaps many more.  This is important because children with different types of arthritis do not have the same prognosis (long term outcome) or best therapy.  If you have been told only that your child has JRA you need to get much more information.  Many of the answers you need can be found below, but much more information is available in my book (It’s not just growing pains click here for more information).

Arthritis in Childhood and Adolescence

 This page is provided by Thomas J. A. Lehman MD

 Delivering the best care - with great care

 Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with SLE.  He practices in New York City.  Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

If you want a more detailed discussion click here for information regarding a book I have written.

Arthritis in childhood? Isn't that only an old person's disease?

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 wheelchair 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 JRA which are separated by how they begin. Pauciarticular 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 antiinflammatory drugs, but it can cause two important problems. 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 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 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 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 JRA 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. This may require use of some fairly strong medications, but it's important. 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 it's 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 have to put a child on steroids we want to get them off as quickly as possible. Nonsteroidal antiinflammatory drugs are enough for many children with polyarticular JRA, but more severe cases may require 'gold shots,' or 'second line' medications like sulphasalazine or methotrexate. Severe cases requiring steroids or 'second line' medications should be under the care of experienced physicians.

The most worrisome form of JRA is systemic onset disease. This form of JRA begins with high fevers and a rash. It is very important in this setting to make sure the patient really has systemic onset JRA 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 JRA. 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 JRA 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 JRA, 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 JRA 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 JRA 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 spondylo-arthropathies as being 'different' since the middle 1970's so good long term follow-up data is not 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 wheelchair. Everyone 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.  The earlier your child gets the proper diagnosis and care the better the results will be.

There are three important things for every child or adolescent with arthritis. First is proper recognition and diagnosis of the disease. Second is proper treatment by an experienced physician with a multidisciplinary support including physical and occupational therapists and orthopedic surgeons. Third is proper education of the patient and family. People with arthritis are no different from everyone else in the world. They all need to grow up, have jobs, get married and have families. 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 or military officers, but even fewer will be 'totally disabled' by their disease. I've seen far more children who were disabled because they were told they couldn't do things than I have who were really disabled by their disease. We should never accept a child with arthritis being told to use a wheelchair. In almost every case we ought to be able to correct the problem and get them walking again. This is why educating the public and physicians is so important. We all need to be aware that children can get arthritis and must be properly diagnosed and treated. But once that's been done we need to make sure they reach their full potential. We all must remember to treat children with arthritis 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.

For more information you may wish to consult my book

 

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Reviewer’s comments

    Dr. Tom Lehmans experience and compassion are evident on every page of this book, and they help guide the readerchild, parent, and healthcare professional alike through the world of childhood arthritis.  This book is an absolute gem written with a single goal in mind:  improve the lives of kids with arthritis. -- Jack Klippel, M.D. President and CEO of the Arthritis Foundation

     Dr. Lehman has given parents and families of children with arthritis the first book that speaks to the parent and child as equals.  His book explains the illnesses, the medications, the lab tests, and the disease course in simple, understandable lay language and givens them valuable insight into how a pediatric rheumatologist thinks.  Bravo!-- Charles Spencer, M.D., Professor of Clinical Pediatrics, University of Chicago, La Rabida

 

It’s not just growing pains.
A guide to childhood muscle, bone, and joint pain,
rheumatic diseases and the latest treatments

 

Click here to see the table of contents

 

It has always been a frustration trying to answer the many questions I have received from people over the web.  I can’t take the time and give them the detail I would like to.  I have to take care of my patients.  This book is a distillation of my experience answering questions for parents and health professionals over 25 years of practice.  If you want to know about the diseases, the tests, the medications, or how to be sure you are getting the best care– If you are the family member of a child with joint pains, this book will give you the answers.  If you are a general physician, a pediatrician, or a nurse who cares for children with these diseases it will answer many of the questions families ask you, and you can recommend it to them.  It will also answer many of your questions about what shots to give, what precautions to take, and the other questions families, pediatricians, and other health care providers have asked me over the years.

 

Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with rheumatic diseases including SLE, JRA, dermatomyositis, scleroderma, Kawasaki disease and related conditions.  He practices in New York City.  Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

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Click here for BOOKS on childhood arthritis

 

This site provided by Thomas J. A. Lehman MD
Chief, Division of Pediatric Rheumatology
The Hospital for Special Surgery
535 E 70 St,
New York, NY 10021
212-606-1151, fax 212-606-1938, e-mail goldscout@aol.com

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