JUVENILE RHEUMATOID ARTHRITIS
What
Is Arthritis?
Arthritis means
joint inflammation, and refers to a group of diseases that cause
pain, swelling, stiffness and loss of motion in the joints.
"Arthritis" is often used as a more general term to refer to
the more than 100 rheumatic diseases that may affect the joints
but can also cause pain, swelling, and stiffness in other supporting
structures of the body such as muscles, tendons, ligaments,
and bones. Some rheumatic diseases can affect other parts of
the body, including various internal organs. Children can develop
almost all types of arthritis that affect adults, but the most
common type of arthritis that affects children is juvenile rheumatoid
arthritis.
What Is Juvenile Rheumatoid Arthritis?
Juvenile rheumatoid
arthritis (JRA) is arthritis that causes joint inflammation
and stiffness for more than 6 weeks in a child of 16 years of
age or less. Inflammation causes redness, swelling, warmth,
and soreness in the joints, although many children with JRA
do not complain of joint pain. Any joint can be affected and
inflammation may limit the mobility of affected joints.
Doctors classify
three kinds of JRA by the number of joints involved, the symptoms,
and the presence or absence of certain antibodies in the blood.
(Antibodies are special proteins made by the immune system.)
These classifications help the doctor determine how the disease
will progress.
- Pauciarticular
(paw-see-are-tick-you-lar): Pauciarticular means
that four or fewer joints are affected. Pauciarticular is
the most common form of JRA; about half of all children with
JRA have this type. Pauciarticular disease typically affects
large joints, such as the knees. Girls under age 8 are most
likely to develop this type of JRA.
Some children have special proteins in the blood called antinuclear
antibodies (ANAs). Eye disease affects about 20 to 30 percent
of children with pauciarticular JRA. Up to 80 percent of those
with eye disease also test positive for ANA and the disease
tends to develop at a particularly early age in these children.
Regular examinations by an ophthalmologist (a doctor who specializes
in eye diseases) are necessary to prevent serious eye problems
such as iritis (inflammation of the iris) or uveitis (inflammation
of the inner eye, or uvea). Many children with pauciarticular
disease outgrow arthritis by adulthood, although eye problems
can continue and joint symptoms may recur in some people.
- Polyarticular:
About 30 percent of all children with JRA have polyarticular
disease. In polyarticular disease, five or more joints are
affected. The small joints, such as those in the hands and
feet, are most commonly involved, but the disease may also
affect large joints. Polyarticular JRA often is symmetrical,
that is, it affects the same joint on both sides of the body.
Some children with polyarticular disease have a special kind
of antibody in their blood called IgM rheumatoid factor (RF).
These children often have a more severe form of the disease,
which doctors consider to be the same as adult rheumatoid
arthritis.
- Systemic:
Besides joint swelling, the systemic form of JRA is characterized
by fever and a light pink rash, and may also affect internal
organs such as the heart, liver, spleen, and lymph nodes.
Doctors sometimes call it Still's disease. Almost all children
with this type of JRA test negative for both RF and ANA. The
systemic form affects 20 percent of all children with JRA.
A small percentage of these children develop arthritis in
many joints and can have severe arthritis that continues into
adulthood.
How Is Juvenile
Rheumatoid Arthritis Different From Adult Rheumatoid Arthritis?
The main difference
between juvenile and adult rheumatoid arthritis is that many
people with JRA outgrow the illness, while adults usually have
lifelong symptoms. Studies estimate that by adulthood, JRA symptoms
disappear in more than half of all affected children. Additionally,
unlike rheumatoid arthritis in an adult, JRA may affect bone
development as well as the child's growth.
Another difference
between JRA and adult rheumatoid arthritis is the percentage
of people who are positive for RF. About 70 to 80 percent of
all adults with rheumatoid arthritis are positive for RF, but
fewer than half of all children with rheumatoid arthritis are
RF positive. Presence of RF indicates an increased chance that
JRA will continue into adulthood.
What Causes Juvenile Rheumatoid Arthritis?
JRA is an autoimmune
disorder, which means that the body mistakenly identifies some
of its own cells and tissues as foreign. The immune system,
which normally helps to fight off harmful, foreign substances
such as bacteria or viruses, begins to attack healthy cells
and tissues. The result is inflammation-marked by redness, heat,
pain, and swelling. Doctors do not know why the immune system
goes awry in children who develop JRA. Scientists suspect that
it is a two-step process. First something in a child's genetic
makeup gives them a tendency to develop JRA; and then an environmental
factor, such as a virus, triggers the development of JRA.
What Are the Symptoms and Signs of Juvenile Rheumatoid
Arthritis?
The most common
symptom of all types of JRA is persistent joint swelling, pain,
and stiffness that typically is worse in the morning or after
a nap. The pain may limit movement of the affected joint although
many children, especially younger ones, will not complain of
pain. JRA commonly affects the knees and joints in the hands
and feet. One of the earliest signs of JRA may be limping in
the morning because of an affected knee. Besides joint symptoms,
children with systemic JRA have a high fever and a light pink
rash. The rash and fever may appear and disappear very quickly.
Systemic JRA also may cause the lymph nodes located in the neck
and other parts of the body to swell. In some cases (less than
half), internal organs including the heart, and very rarely,
the lungs may be involved.
Eye inflammation
is a potentially severe complication that sometimes occurs in
children with pauciarticular JRA. Eye diseases such as iritis
and uveitis often are not present until some time after a child
first develops JRA.
Typically, there
are periods when the symptoms of JRA are better or disappear
(remissions) and times when symptoms are worse (flares). JRA
is different in each child—some may have just one or two
flares and never have symptoms again, while others experience
many flares or even have symptoms that never go away.
Does Juvenile Rheumatoid Arthritis Affect Physical
Appearance?
Some children with
JRA may look different because they have growth problems. Depending
on the severity of the disease and the joints involved, growth
in affected joints may be too fast or too slow, causing one
leg or arm to be longer than the other. Overall growth may also
be slowed. Doctors are exploring the use of growth hormones
to treat this problem. JRA also may cause joints to grow unevenly
or to one side.
Children with JRA
also may look different because of medication. Corticosteroids,
a type of medication sometimes used to treat JRA, can result
in weight gain and a round face. When the doctor stops giving
the medication, these side effects may disappear.
How Is Juvenile Rheumatoid Arthritis Diagnosed?
Doctors usually
suspect JRA, along with several other possible conditions, when
they see children with persistent joint pain or swelling, unexplained
skin rashes and fever, or swelling of lymph nodes or inflammation
of internal organs. A diagnosis of JRA also is considered in
children with an unexplained limp or excessive clumsiness.
No one test can
be used to diagnose JRA. A doctor diagnoses JRA by carefully
examining the patient and considering the patient's medical
history and the results of laboratory tests that help rule out
other conditions.
- Symptoms:
One important consideration in diagnosing JRA is the length
of time that symptoms have been present. Joint swelling or
pain must last for at least 6 weeks for the doctor to consider
a diagnosis of JRA. Because this factor is so important, it
may be useful to keep a record of the symptoms, when they
first appeared, and when they are worse or better.
- Laboratory
Tests: Laboratory tests, usually blood tests, cannot by
themselves provide the doctor with a clear diagnosis. But
these tests can be used to help rule out other conditions
and to help classify the type of JRA that a patient has. Blood
may be taken to test for RF or ANA, and to determine the erythrocyte
sedimentation rate (ESR).
- ANA is found
in the blood more often than RF, and both are found in
only a small portion of JRA patients. The RF test helps
the doctor tell the difference among the three types of
JRA.
- ESR is a
test that measures how quickly red blood cells fall to
the bottom of a test tube. Some people with rheumatic
disease have an elevated ESR or "sed rate" (cells fall
quickly to the bottom of the test tube), showing that
there is inflammation in the body. Not all children with
active joint inflammation have an elevated ESR.
- X Rays: X
rays are needed if the doctor suspects injury to the bone
or unusual bone development. Early in the disease, some x
rays can show cartilage damage. In general, x rays are more
useful later in the disease, when bones may be affected.
- Other diseases:
Because there are many causes of joint pain and swelling,
the doctor must rule out other conditions before diagnosing
JRA. These include physical injury, bacterial infection, Lyme
disease, inflammatory bowel disease, lupus, dermatomyositis,
and some forms of cancer. The doctor may use additional laboratory
tests to help rule out these and other possible conditions.
Who Treats Juvenile
Rheumatoid Arthritis? What Are the Treatments?
A pediatrician,
family physician, or other primary care doctor frequently manages
the treatment of a child with JRA, often with the help of other
doctors. Depending on the patient's and parents' wishes and
the severity of the disease, the team of doctors may include
pediatric rheumatologists (doctors specializing in childhood
arthritis), ophthalmologists (eye doctors), orthopaedic surgeons
(bone specialists), and physiatrists (rehabilitation specialists),
as well as physical and occupational therapists.
The main goals of
treatment are to preserve a high level of physical and social
functioning and maintain a good quality of life. To achieve
these goals, doctors recommend treatments to reduce swelling;
maintain full movement in the affected joints; relieve pain;
and identify, treat, and prevent complications. Most children
with JRA need medication and physical therapy to reach these
goals.
Several types
of medication are available to treat JRA:*
*Brand names
included in this fact sheet are provided as examples only, and
their inclusion does not mean that these products are endorsed
by the National Institutes of Health or any other Government
agency. Also, if a particular brand name is not mentioned, this
does not mean or imply that the product is unsatisfactory.
- Nonsteroidal
anti-inflammatory drugs (NSAIDs): Aspirin, ibuprofen (Motrin,
Advil, Nuprin) and naproxen or naproxen sodium (Naprosyn,
Aleve) are examples of NSAIDs. They often are the first type
of medication used. Most doctors do not treat children with
aspirin because of the possibility that it will cause bleeding
problems, stomach upset, liver problems, or Reye's syndrome.
But for some children, aspirin in the correct dose (measured
by blood test) can control JRA symptoms effectively with few
serious side effects.
If the doctor prefers not to use aspirin, other NSAIDs are
available. For example, in addition to those mentioned above,
diclofenac and tolmetin are available with a doctor's prescription.
Studies show that these medications are as effective as aspirin
with fewer side effects. An upset stomach is the most common
complaint. Any side effects should be reported to the doctor,
who may change the type or amount of medication.
- Disease-modifying
anti-rheumatic drugs (DMARDs): If NSAIDs do not relieve
symptoms of JRA, the doctor is likely to prescribe this type
of medication. DMARDs slow the progression of JRA, but because
they take weeks or months to relieve symptoms, they often
are taken with an NSAID. Various types of DMARDs are available.
In the past, doctors prescribed hydroxychloroquine, oral and
injectable gold, sulfasalazine, and d-penicillamine; however,
doctors are now much more likely to use methotrexate for children
with JRA.
- Methotrexate:
Researchers have learned that this type of DMARD is safe and
effective for some children with rheumatoid arthritis whose
symptoms are not relieved by other medications. Because only
small doses of methotrexate are needed to relieve arthritis
symptoms, potentially dangerous side effects rarely occur.
The most serious complication is liver damage, but it can
be avoided with regular blood screening tests and doctor followup.
Careful monitoring for side effects is important for people
taking methotrexate. When side effects are noticed early,
the doctor can reduce the dose and eliminate side effects.
- Corticosteroids:
In children with very severe JRA, stronger medicines may be
needed to stop serious symptoms such as inflammation of the
sac around the heart (pericarditis). Corticosteroids like
prednisone may be added to the treatment plan to control severe
symptoms. This medication can be given either intravenously
(directly into the vein) or by mouth. Corticosteroids can
interfere with a child's normal growth and can cause other
side effects, such as a round face, weakened bones, and increased
susceptibility to infections. Once the medication controls
severe symptoms, the doctor may reduce the dose gradually
and eventually stop it completely. Because it can be dangerous
to stop taking corticosteroids suddenly, it is important that
the patient carefully follow the doctor's instructions about
how to take or reduce the dose.
In addition to medications,
physical therapy is an important part of a child's treatment
plan. Exercise can help to maintain muscle tone and preserve
and recover the range of motion of the joints. A physical therapist
can design an appropriate exercise program for a person with
JRA. The physical therapist also may recommend using splints
and other devices to keep joints growing evenly.
How Can the Family
Help a Child Live Well With JRA?
JRA affects the
entire family who must cope with the special challenges of this
disease. JRA can strain a child's participation in social and
after-school activities and make school work more difficult.
There are several things that family members can do to help
the child do well physically and emotionally.
- Treat the child
as normally as possible.
- Ensure that the
child receives appropriate medical care and follows the doctor's
instructions. Many treatment options are available, and because
JRA is different in each child, what works for one may not
work for another. If the medications that the doctor prescribes
do not relieve symptoms or if they cause unpleasant side effects,
patients and parents should discuss other choices with their
doctor. A person with JRA can be more active when symptoms
are controlled.
- Encourage exercise
and physical therapy for the child. For many young people,
exercise and physical therapy play important roles in treating
JRA. Parents can arrange for children to participate in activities
that the doctor recommends. During symptom-free periods, many
doctors suggest playing team sports or doing other activities
to help keep the joints strong and flexible and to provide
play time with other children and encourage appropriate social
development.
- Work closely
with the school to develop a suitable lesson plan for the
child and to educate the teacher and the child's classmates
about JRA. (See the end of this fact sheet for information
about Kids on the Block, Inc., a program
that uses puppets to illustrate how juvenile arthritis can
affect school, sports, friends, and family.) Some children
with JRA may be absent from school for prolonged periods and
need to have the teacher send assignments home. Some minor
changes such as an extra set of books, or leaving class a
few minutes early to get to the next class on time can be
a great help. With proper attention, most children progress
normally through school.
- Explain to the
child that getting JRA is nobody's fault. Some children believe
that JRA is a punishment for something they did.
- Consider joining
a support group. The American Juvenile Arthritis Organization
runs support groups for people with JRA and their families.
Support group meetings provide the chance to talk to other
young people and parents of children with JRA and may help
a child and the family cope with the condition.
Do Children With
Juvenile Rheumatoid Arthritis Have To Limit Activities?
Although pain sometimes
limits physical activity, exercise is important to reduce the
symptoms of JRA and maintain function and range of motion of
the joints. Most children with JRA can take part fully in physical
activities and sports when their symptoms are under control.
During a disease flare, however, the doctor may advise limiting
certain activities depending on the joints involved. Once the
flare is over, a child can start regular activities again.
Swimming is particularly
useful because it uses many joints and muscles without putting
weight on the joints. A doctor or physical therapist can recommend
exercises and activities.
What Are Researchers Trying To Learn About Juvenile
Rheumatoid Arthritis?
Scientists are investigating
the possible causes of JRA. Researchers suspect that both genetic
and environmental factors are involved in development of the
disease and they are studying these factors in detail. To help
explore the role of genetics, the National Institute of Arthritis
and Musculoskeletal and Skin Diseases (NIAMS) has established
a research registry for families in which two or more siblings
have JRA. NIAMS also funds a Multipurpose Arthritis and Musculoskeletal
Diseases Center (MAMDC) that specializes in research on pediatric
rheumatic diseases including JRA.
Research doctors
are continuing to try to improve existing treatments and find
new medicines that will work better with fewer side effects.
For example, researchers are studying the long-term effects
of the use of methotrexate in children.
Where Can People Get More Information About the
Research Registry and MAMDC?
For more information
about the Research Registry, contact:
Edward Giannini, MD
Children's Hospital Medical Center - PAV 2-129
University of Cincinnati, College of Medicine
Cincinnati, OH 45229
513/636-7634 or 513/636-4495
E-mail address: btague@one.net
World Wide Web address: http://www.jraregistry.org
For information
about the MAMDC, contact:
David Glass, MD
Children's Hospital Medical Center - PAV 2-129
University of Cincinnati, College of Medicine
Cincinnati, OH 45229-2899
513/636-8854
E-mail address: glasd0@chmcc.org
World Wide Web address: http://www.cinciMAMDC.org
Where Can People Get More Information About Juvenile
Rheumatoid Arthritis?
- American Juvenile
Arthritis Organization (AJAO)
1330 West Peachtree Street
Atlanta, GA 30309
404/872-7100
800/283-7800
World Wide Web address: http://www.arthritis.org/
AJAO, part of the National Arthritis Foundation, is the
primary nonprofit organization devoted to childhood rheumatic
diseases. The organization has information about JRA, support
groups, and pediatric rheumatology centers around the country.
- National Institute
of Arthritis and Musculoskeletal and Skin Diseases Information
Clearinghouse
National Institutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
301/495-4484 Fax: 301/587-4352 TTY: 301/565-2966
NIAMS Fast Facts: health information available 24 hours a
day by fax, call 301/881-2731 from a fax machine telephone.
World Wide Web address: http://www.nih.gov/niams
National Institute of Arthritis and Musculoskeletal and Skin
Diseases Information Clearinghouse is a public service sponsored
by the NIAMS that provides health information devoted to childhood
rheumatic diseases. The organization has information about
JRA, support groups, and pediatric rheumatology centers around
the country.
- Kids
on the Block, Inc.
9385-C Gerwig Lane
Columbia, MD 21046
410/290-9095
800/368-KIDS (5437)
Kids on the Block, Inc., is an educational program that uses
puppets to show how JRA can affect school, sports, friends,
and family. A package is available (for a fee) that includes
a set of large puppets that represent a diverse group of children,
as well as audiocassettes, a training guide, four different
program scripts, props, followup activities, and other resources.
The program is designed so that anyone can be puppeteer, and
workshops to train puppeteers are available.
Acknowledgments
The NIAMS gratefully
acknowledges the assistance of Lauren Pachman, M.D., of Children's
Hospital, Chicago, IL; Patience White, M.D., of George Washington
Medical Center and Children's National Medical Center, Washington,
DC; and Edward H. Giannini, M.D., of Children's Hospital Medical
Center at the University of Cincinnati.
The National Arthritis
and Musculoskeletal and Skin Diseases Information Clearinghouse
(NAMSIC) is a public service sponsored by the NIAMS that provides
health information and information sources. The NIAMS, a part
of the National Institutes of Health (NIH), leads the Federal
medical research effort in arthritis and musculoskeletal and
skin diseases. The NIAMS sponsors research and research training
throughout the United States as well as on the NIH campus in
Bethesda, MD, and disseminates health and research information.
Publication number: AR-112QA
Publication date: May 1998
E-text posted: October 1998
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