|Questions and Answers About Juvenile
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 that affects children is
juvenile rheumatoid arthritis (JRA).
What Is Juvenile Rheumatoid Arthritis?
Juvenile rheumatoid arthritis 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. One type of JRA can also affect the
internal organs. Doctors classify JRA into three types by the number of
joints involved, the symptoms, and the presence or absence of certain
antibodies found by a blood test. (Antibodies are special proteins made
by the immune system.) These classifications help the doctor determine
how the disease will progress and whether the internal organs or skin is
- 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
Some children have special kinds of antibodies in the
blood. One is called antinuclear antibody (ANA) and one is called
rheumatoid factor. 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, the colored part of the eye) or uveitis (inflammation of
the uvea, or the inner eye). Some 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 an 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 similar in many ways to
adult rheumatoid arthritis.
- Systemic--Besides joint swelling, the systemic form of JRA
is characterized by fever and a light skin 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.
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; 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 skin 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
(flare-ups). JRA is different in each child--some may have just one or
two flare-ups and never have symptoms again, while others experience
many flare-ups or even have symptoms that never go away.
Some children with JRA may 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.
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
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, the results of laboratory tests, and x rays
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 and ANA, and to determine the erythrocyte sedimentation
- 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 or viral
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
Who Treats Juvenile Rheumatoid Arthritis?
What Are the Treatments?
The special expertise of rheumatologists in caring for
patients with JRA is extremely valuable. Pediatric rheumatologists are
trained in both pediatrics and rheumatology and are best equipped to
deal with the complex problems of children with arthritis and other
rheumatic diseases. However, there are very few such specialists, and
some areas of the country have none at all. In such circumstances, a
team approach involving the child's pediatrician and a rheumatologist
with experience in both adult and pediatric rheumatic disease provides
optimal care for children with arthritis. Other important members of the
team include physical therapists 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
- 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
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. Doctors are
likely to use one type of DMARD, methotrexate, for children with
Researchers have learned that methotrexate 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.
- Biologic agents--Children with polyarticular JRA who have
gotten little relief from other drugs may be given one of a new class
of drug treatments called "biologic agents." Etanercept (Enbrel), for
example, is such an agent. It blocks the actions of tumor necrosis
factor, a naturally occurring protein in the body that helps cause
- Physical therapy--Exercise is an important part of a
child's treatment plan. It can help to maintain muscle tone and
preserve and recover the range of motion of the joints. A physiatrist
(rehabilitation specialist) or a physical therapist can design an
appropriate exercise program for a person with JRA. The specialist
also may recommend using splints and other devices to help maintain
normal bone and joint growth.
- Complementary and alternative medicine--Many adults seek
alternative ways of treating arthritis, such as special diets or
supplements. Although these methods may not be harmful in and of
themselves, no research to date shows that they help. Some people have
tried acupuncture, in which thin needles are inserted at specific
points in the body. Others have tried glucosamine and chondroitin
sulfate, two natural substances found in and around cartilage cells,
for osteoarthritis of the knee.
Some alternative or
complementary approaches may help a child to cope with or reduce some
of the stress of living with a chronic illness. If the doctor feels
the approach has value and will not harm the child, it can be
incorporated into the treatment plan. However, it is important not to
neglect regular health care or treatment of serious symptoms.
* Brand names included in this booklet 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.
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
- Encourage exercise and physical therapy for the child. For many
young people, exercise and physical therapy play important roles in
managing 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 booklet 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
- 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.
- Work with therapists or social workers to adapt more easily to the
lifestyle change JRA may bring.
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-up, however, the doctor may advise
limiting certain activities depending on the joints involved. Once the
flare-up 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.
The research registry for JRA is located at Children's
Hospital Medical Center at the University of Cincinnati College of
Medicine in Ohio. The registry, established in 1994, continues to list
new cases as well as be maintained and systematically updated. The focus
of the registry is on families whose brothers and sisters have JRA, with
emphasis on genetic susceptibility in those affected families.
Researchers 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. In addition, the Food and Drug
Administration's "Pediatric Rule" requires manufacturers of new drugs
and biologic agents, such as etanercept, that will be commonly used for
children to provide specific information about safe pediatric use.
Where Can People Get More Information About the MAMDC?
For more information about the MAMDC, contact:
David Glass, M.D.
Children's Hospital Medical
University of Cincinnati, College of
Cincinnati, OH 45229-2899
Phone: 513-636-8854 or
800-559-7001 (free of charge)
Where Can People Get More Information About Juvenile Rheumatoid Arthritis?
National Institute of Arthritis and Musculoskeletal and
Skin Diseases (NIAMS)
National Institutes of Health
Bethesda, MD 20892-3675
Phone: 301-495-4484 or 877-22-NIAMS
(226-4267) (free of charge)
NIAMS provides information about various forms of
arthritis and rheumatic disease and bone, muscle, joint, and skin
diseases. It distributes patient and professional education materials
and refers people to other sources of information. Additional
information and updates can also be found on the NIAMS Web site.
American Academy of Orthopaedic Surgeons
Des Plaines, IL 60017
Phone: 800-824-BONE (2663) (free of
The academy publishes brochures on arthritis and other
subjects. Single copies of a brochure are available free of charge by
sending a self-addressed, stamped (business-size) envelope to (name of
brochure) at the address above.
American College of Rheumatology
Place, Suite 250
Atlanta, GA 30345
The association provides referrals to doctors and health
professionals who work on arthritis, rheumatic diseases, and related
conditions. The association also provides educational materials and
American Juvenile Arthritis Organization
West Peachtree Street
Atlanta, GA 30309
Phone: 404-872-7100 or
800-283-7800 (free of charge)
Part of the National Arthritis Foundation, this
organization is the primary nonprofit group devoted to childhood
rheumatic diseases. It has information about JRA, support groups, and
pediatric rheumatology centers around the country.
Kids on the Block, Inc.
Columbia, MD 21046
Phone: 410-290-9095 or 800-368-KIDS (5437)
(free of charge)
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 a puppeteer, and workshops to train puppeteers are
NIH Publication No. 01-4942