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.
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
My book –click here to order at a discount from Amazon.com!!
at
Amazon.com (only $24.50)
Reviewer’s
comments
“Dr. Tom Lehman’s experience and
compassion are evident on every page of this book, and they help guide the
reader—child, 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.
Click here if you are interested in
making an appointment with Dr. Lehman
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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