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.
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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.
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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.
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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.
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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.
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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).
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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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.
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Treat the child
as normally as possible.
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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.
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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.
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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.
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Explain to the
child that getting JRA is nobody's fault. Some children believe that JRA is a
punishment for something they did.
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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 MAMDC?
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
Where Can People Get More Information About Juvenile Rheumatoid
Arthritis?
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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.
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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
World Wide Web address: http://www.niams.nih.gov/
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.
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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.
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