Topic Overview
What is juvenile idiopathic arthritis?
Juvenile idiopathic arthritis (JIA) is a childhood disease that causes
inflamed, swollen joints. This makes joints stiff and painful. The term "juvenile idiopathic arthritis" is replacing the American "juvenile rheumatoid arthritis" and the European "juvenile chronic arthritis."
Some children with the disease
grow out of it after they get treatment. Others will need ongoing treatment as
adults.
There are several types of juvenile idiopathic
arthritis.
-
Oligoarticular
(formerly known as pauciarticular) is the most common and
often the mildest type. Your child may have pain in 1 to 4 joints during the first 6 months of the disease, such as the knees,
ankles, fingers, toes, wrists, elbows, or hips. The condition is called persistent oligoarthritis if no more than 4 joints are involved, and extended oligoarthritis if more joints become involved in the first 6 months.
-
Polyarticular
affects more joints
and tends to get worse over time. It is further divided by whether or not the child has an antibody called rheumatoid factor in their blood. If the antibody is present, the condition is more severe, and is more like rheumatoid arthritis in adults.
-
Systemic
is less common, but it can be the most serious. It causes pain in many
joints and can also spread to organs.
-
Enthesitis-related
is also less common than oligoarticular and polyarticular JIA. It most often affects the areas where tendons and ligaments attach to bones (the enthesis). The joints may also be affected.
- Psoriatic usually combines joint tenderness and inflammation (arthritis) with psoriasis of the skin or a related condition of the nails.
What causes juvenile idiopathic arthritis?
Doctors don't really know what causes the
disease. But there are a number of things that they think can lead to it. These
things include:
- An
immune system that is too active and attacks joint
tissues.
- Viruses or other infections that cause the immune system
to attack joint tissues.
- Having certain
genes that make the immune system more likely to
attack joint tissues.
What are the symptoms?
Children can have one or many symptoms, such as:
- Joint pain.
- Joint
swelling.
- Joint stiffness.
- Trouble
sleeping.
- Problems walking.
- Fever.
- Rash.
In some cases these symptoms can be mild and hard for you
to see. A young child may be more cranky than normal or may go back to crawling
after he or she has started walking. You may notice that your child feels stiff
in the morning or has trouble walking.
Children with this disease
can also get inflammatory
eye disease. This can lead to permanent vision
problems or blindness if it’s not treated. Eye disease often has no symptoms
before vision loss occurs. That’s why it’s important for your child to have
regular eye exams with an
ophthalmologist. Treatment can begin before your child
has long-lasting vision problems.
How is juvenile idiopathic arthritis diagnosed?
Your doctor will ask
questions about your child’s symptoms and past health and will do a physical
exam. Your child may also have blood tests and a urine test to look for signs
of the disease. If your child has the disease, these tests can help your doctor
find out which type it is.
How is it treated?
Your child’s treatment will be
based on the type of arthritis he or she has and how serious it is. The most
common treatment includes medicines to reduce pain and swelling
(NSAIDs), along with
physical therapy and often occupational therapy. Your child may also get shots of
steroid medicine into a joint to relieve swelling and
pain.
If these treatments don't help, then your child may be given
other medicines. Surgery to correct joint problems is only done in rare
cases.
Exercise is an important part of your child’s treatment.
Physical therapists can teach you and your child exercises to keep your child’s
muscles flexible and strong. Moving your child's painful joints through their
full range of motion keeps them from getting stiff or deformed. Many children
with the disease don't want to move painful joints. Your child may need your
help to keep doing daily physical therapy.
Even when juvenile
idiopathic arthritis is not a severe type, your child may still need long-term
treatment. To make sure that treatment is right for your child, work closely
with the medical team. Learn as much as you can about your child’s disease and
treatments. Stay on a schedule with your child’s medicines and exercise.
How do you cope with juvenile idiopathic arthritis?
Exercise, medicine, and assistive devices will
help your child get through each day as normally as possible. Assistive devices
are things that can help your child hold onto, open, or close things more
easily. A doorknob extender, used to open a door without twisting a wrist, is
one such device.
Children who have this disease need to balance
exercise and rest. They may need extra rest during the day to relax their
joints and keep up their energy. But be sure that your child gets enough
exercise. This will help keep joints strong and flexible.
Pain
relief exercises can help you and your child control joint pain caused by the
disease. Your child's doctor can help you set up a pain management plan. This
plan might include heat treatments, exercise, and a type of counseling called
cognitive-behavioral therapy. Breathing and relaxation
exercises can also help ease your child’s pain.
Frequently Asked Questions
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Cause
The cause of
juvenile idiopathic arthritis (JIA) is not well
understood. Most experts believe it is caused by a combination of factors,
including:
- An overly active
immune system that inappropriately attacks joint
tissues, as though they were a foreign substance.
- Viral or
bacterial infections, which are a suspected trigger of the
autoimmune process.
-
Genetic factors
that make a child's immune system more
likely to react inappropriately. A study of relatives of children with JIA
reported a higher occurrence of other autoimmune diseases in these families. These families may share genes that make them more susceptible to
autoimmune diseases, including JIA.
Most experts and health professionals are now
referring to this condition as juvenile idiopathic arthritis (JIA): idiopathic means "of
unknown cause." As the international terminology becomes more widely used, you
may hear different terms used to describe each type of childhood arthritis. To
learn more about the new international "juvenile idiopathic arthritis"
classification, as compared with the American "juvenile rheumatoid arthritis"
and the European "juvenile chronic arthritis," see
classification.
Symptoms
The most common symptoms of all forms of
juvenile idiopathic arthritis (JIA) include:
- Joint pain and swelling that may come and go
but are most often persistent.
- Joint stiffness in the
morning.
- Irritability, refusal to walk, or protection or guarding
of a joint. You might notice your child limping or trying not to use a certain
joint.
- Often unpredictable changes in symptoms, from periods with no
symptoms (remission) to flare-ups.
Even though pain is a common symptom of JIA, a child may
not identify pain as a problem. A child may be unable to describe pain or may
become accustomed to the presence of pain. The child may be more alarmed by
symptoms such as stiffness and may be better able to describe those symptoms.
Some researchers believe that some children who have repeated medical
procedures that cause pain may be afraid of further doctor visits and more
anxious about their illness.1 This could cause them to
not express or identify their pain because of fear of medical
procedures.
Additional symptoms vary depending on
which type of JIA a child has.
Eye disease usually
causes no symptoms before permanent vision loss occurs. For this reason, it is
very important for a child who has JIA to have eye examinations with an
ophthalmologist to detect developing eye damage so
that treatment can be started before permanent vision problems occur. If
symptoms are present, they may be as mild as painless red eyes, or the symptoms
can include blurred vision, eye pain, sensitivity to light, and vision
loss.
Fever spikes caused by systemic JIA typically reach
103°F (39.5°C) to
106°F (41°C), one to two times
daily, with a fall to normal between spikes.
Rash caused by
systemic JIA is spotty, flat, and sometimes faint red or pink and may occur
with the fever. It may erupt over the torso, face, palms, soles of feet, and
armpits. The rash often comes and goes and may appear late in the day or in the
early morning. It may also be brought on by warm baths or by rubbing or
scratching the skin.
Other conditions with symptoms similar
to JIA include
growing pains, overuse, injury, bone infection, and
certain inflammatory diseases, among others. Many conditions can cause painful,
stiff joints in children. Most often, occasional joint pain in children is
related to an injury or aggravating factors, such as repetitive overuse in
sports activities. JIA is a relatively uncommon cause of these symptoms.
Some children have forms of chronic arthritis that are similar to
yet distinct from juvenile idiopathic arthritis. These conditions, called
spondyloarthropathies, are not addressed in this
topic. Examples of spondyloarthropathies are
ankylosing spondylitis,
Reiter's syndrome, and
psoriatic arthritis.
What Happens
The course of
juvenile idiopathic arthritis (JIA) is unpredictable,
especially during the first few years after a child is diagnosed. JIA, also
called juvenile rheumatoid arthritis (JRA) or juvenile chronic arthritis (JCA),
can be mild, causing few problems. Symptoms can get worse or disappear without
clear reason. Eventually the pattern of symptoms becomes more predictable. In
general, children with JIA have one or a combination of symptoms including
joint pain, joint swelling, and joint stiffness early in the course of the
disease. Many children experience sleep disturbances, including difficulty
falling asleep and frequent night awakenings.2 Most
children have good and bad days.
Of all children who have JIA, 3 or 4
out of 10 children will have long-term disability.3
While the overall long-term outlook for children with JIA is often good,
symptoms of the disease can continue into adulthood. Long-term disability may
range from occasional stiffness, the need for pain medicine, and limits on
physical activity to ongoing arthritis and the need for major surgery such as
joint replacement. But for most adults who had JIA as children, any long-term
problems tend to be mild and do not affect their overall quality of life. For
instance, they may not be able to play certain sports, but their activities are
not otherwise limited.
A child's long-term outlook is influenced
by the type of juvenile idiopathic arthritis he or she has. While a child with
oligoarticular JIA (4 or fewer joints affected) has a
good long-term outlook other than eye disease risk, a child with
polyarticular JIA (5 or more joints) or
systemic JIA (whole-body symptoms) is likely to have
more long-term problems.4
Treatment also affects the child's long-term outlook. Treatment is usually started as soon as the child is diagnosed, because if treatment can control the inflammation, there is less long-term disability and the tissues may heal over time.4
Oligoarticular JIA (oligoarthritis)
Up to
60% of all children affected by JIA have the oligoarticular form.4 Some children with more severe disease have joint damage that
shows on X-rays within 5 years. Children with oligoarticular JIA
(oligoarthritis, meaning "few joints")
may continue to have disease as an adult. This risk is higher if more joints are affected after the first 6 months (extended). Among children with
oligoarticular JIA:4
- Most children continue to have 4 or fewer joints
affected. This is called persistent oligoarthritis.
- Fewer than half go on to have 5 or more joints affected after the first 6 months of the disease. This is called extended oligoarthritis, resembling
polyarthritis).
- There is an increased risk of having vision loss
caused by
inflammatory eye disease.
- Some children
have uneven leg bone growth, resulting in legs of different length and muscle
wasting.3
Polyarticular JIA (polyarthritis)
Polyarticular
JIA (polyarthritis) affects 5 or more joints in the first 6 months of the disease—often the knee, hip, wrist, elbow, and
ankle joints—and may affect the small joints in the hands and feet. This type
of JIA is more severe than most cases of oligoarticular JIA because it affects more joints
and tends to get worse over time. Joint damage can be seen on X-ray within 2
years in children with more severe disease. About 30% of children
affected by JIA have the polyarticular form.4 Of these children, many will have active disease that continues into adulthood.
Polyarticular JIA is divided into two groups.
- If an antibody called
rheumatoid factor is present in the blood, the polyarthritis is rheumatoid factor-positive (RF-positive).
- If the rheumatoid factor antibody is not present, the polyarthritis is rheumatoid factor-negative (RF-negative).
Of all children with juvenile idiopathic arthritis, fewer than 10% have RF-positive polyarticular JIA.4 Normally, antibodies are produced by the
immune system to help destroy and eliminate invading bacteria and viruses that
can cause disease. But RF is an antibody that can attach to normal body tissue,
resulting in damage. RF-positive polyarticular JIA is thought to be identical
to adult
rheumatoid arthritis. The risk of joint deformity is
highest (about 50% likelihood) in children with RF-positive polyarticular
JIA.5 In RF-positive JIA:
- Some children develop bumps under the skin (rheumatoid nodules) over pressure points (such as the elbow or back of the
heel).
- Children are less likely to have a long-term remission of symptoms, so they have a higher risk of disability as an adult.
RF-negative JIA is much more common than RF-positive JIA. In RF-negative JIA:
Systemic JIA
About 10% of children
affected by JIA have the systemic form.4 Usually, a
child who has systemic JIA will have fever spikes and a rash for weeks to months
before arthritis joint pain begins. Whole-body (systemic) symptoms (such as
fatigue and loss of appetite) and enlarged lymph nodes, liver, and spleen may
come and go during the first years of the disease. Some children
with systemic JIA develop heart and complications.5
Some children with systemic JIA will have joint
damage visible on X-ray within 2 years. Many will continue to have active disease as adults. While some children have one course of this disease that lasts 2 to 4 years, others continue to have mild joint pain and flares of other symptoms. A few have ongoing destructive arthritis, often into adulthood, even with treatment.4
Enthesitis-related JIA
About 10% of children with JIA have the enthesitis-related form. An enthesis is the area where a tendon or ligament attaches to a bone. In enthesitis-related JIA, that area is inflamed and tender. This is most common in the front of the knee and at the heel. In addition to enthesitis, the child has joint pain and inflammation (arthritis), or 2 or more other features such as:4
- Sacroiliac tenderness (where the spine meets the pelvis).
- Low back and buttock pain and inflammation.
- Presence of an antigen called HLA-B27 in the blood.
-
Inflammatory eye disease.
- Family history of another condition related to HLA-B27, such as ankylosing spondylitis.
The long-term outlook for this form of JIA is less well known, but some children progress to other conditions such as adult ankylosing spondylitis. This is more common in boys who have hip arthritis or who have HLA-B27.
Psoriatic JIA
Psoriatic is the least common form, occurring in fewer than 10% of children with JIA. Children with psoriatic arthritis have a skin condition called psoriasis. In addition to psoriasis, they have arthritis of several joints, or they have at least 2 other features, such as:4
- Problem with the finger or toe nails. This can be pitting of the nails, or separation of the nail from the nail bed (onycholysis).
- Family history of psoriasis in a close relative.
These children can also have inflammatory eye disease.
As with enthesitis-related JIA, the long-term outcome for psoriatic JIA is not well known. Some children continue to have skin and joint symptoms. For a few, the ability to do daily activities can become limited.
Complications
Complications associated with JIA
can include:
-
Inflammatory eye disease, such as
uveitis. Children and adults with this condition can
develop
cataracts,
glaucoma,
corneal degeneration (band keratopathy), or vision
loss.
-
Growth abnormalities, such as unequal leg lengths, an
imbalance in growth of the jaw, and temporary delay in breast
growth.
- Joint damage. This is common in the polyarticular form of
JIA and can occur early. About 30% to 50% of children with JIA may have some level of
disability that continues into adulthood.4 Long-term problems can be mild and have little effect on daily activities.
Some children with polyarthritis develop arthritis in the
neck that can cause the neck bones to fuse together.
Complications of systemic JIA include heart or lung problems, such as
pericarditis,
pleuritis, or
pericardial effusion. A rare lung complication is the
formation of scar tissue in the lungs (pulmonary fibrosis).
What Increases Your Risk
No clear risk factors for
juvenile idiopathic arthritis (JIA) are known at this
time. A study of relatives of children with JIA reported a higher
occurrence of other
autoimmune diseases in these families. These families may share genes that make them more susceptible to autoimmune
diseases, including JIA. But more children who have JIA have no family history of the disease.
When To Call a Doctor
Call your doctor immediately if:
- Your child has sudden, unexplained swelling,
redness, and pain in any joint or joints.
- A baby or child is
unusually cranky or reluctant to crawl or walk.
- Red eyes, eye pain,
and vision blurring or loss occur in a child who has been diagnosed with any
form of juvenile arthritis.
Call your doctor if any of the following symptoms continue
for more than 2 days:
- A child has unexplained daily fever spikes
[103°F (39.4°C) to
106°F (41.1°C)] with or without
a pink skin rash.
- A baby or child is reluctant to crawl or walk in
the early morning but improves after 1 to 2 hours.
- A child taking aspirin or another nonsteroidal
anti-inflammatory drug (NSAID) develops stomach pain not clearly related to
stomach flu, but possibly related to medicine use (symptoms such as heartburn,
nausea, or refusal to eat).
- Joint pain and skin rash develop
following a sore throat.
Watchful Waiting
It can be hard to know when an infant has joint
pain. A young child may be unusually cranky or may revert to crawling after he
or she has started walking. You may notice gait problems with a walking child
or stiffness in the morning.
It is reasonable to try home
treatment (hot or cold packs, rest, and acetaminophen) for mild joint pain. If
there is no improvement in 1 to 2 weeks or if any of the other symptoms
described above are present, see a doctor. If redness or swelling is present in
a single joint, or if the pain is severe, call your doctor immediately. This
could mean an infection in the joint.
Who To See
For initial evaluation of joint pain and other
symptoms of
juvenile idiopathic arthritis (JIA), consult with
a:
For additional testing and disease management, consult
with a
rheumatologist who specializes in children's rheumatic
disease (pediatric rheumatologist).
The disease management team
for JIA may also include:
- An
orthopedic surgeon who specializes in children's
orthopedic problems (pediatric
orthopedist).
- Nurses.
- Physical and occupational
therapists.
- A registered dietitian or nutritionist, as
needed.
- A social worker or psychologist, as needed.
- A
general dentist and an orthodontist, as needed.
- An
ophthalmologist.
To prepare for your appointment, see the topic Making the Most of Your Appointment.
Exams and Tests
Findings from a physical examination,
including the pattern and nature of joint symptoms, are important keys to the
diagnosis of
juvenile idiopathic arthritis (JIA). In most cases,
routine lab results do not point to an obvious diagnosis of this disease. JIA
is often diagnosed only after other possible causes of symptoms have been ruled
out and the pain and stiffness have lasted for at least 6 weeks. The following
tests are mainly done to see whether another medical condition is causing joint
pain or whole-body (systemic) symptoms.
Routine examinations and
tests include the following:
The following tests are done if needed:
Early Detection
There are no standard screening tests that are
used to identify children who may develop juvenile idiopathic arthritis
(JIA).
Early eye disease detection
Slit lamp eye examinations are necessary for all
children with juvenile idiopathic arthritis to test for possible eye problems,
such as
uveitis. This test may be repeated often during the
course of the condition because the
inflammatory eye disease associated with JIA generally
has no symptoms and can lead to a permanent decrease in vision or
blindness.
Inflammatory eye disease risk is not related to how
severe a child's other JIA symptoms are. In fact, children at greatest risk are
girls who develop mild oligoarticular disease (oligoarthritis) during their
early childhood years and have developed high levels of antinuclear antibodies
(ANAs).
Treatment Overview
The goals of medical treatment for
juvenile idiopathic arthritis (JIA) are to reduce your
child's joint pain and to prevent disability. Physical therapy and medicine are
the basis of medical treatment for JIA.
Treatment is determined by the type and
severity of JIA. Even when JIA is uncomplicated, an affected child may need years of medical treatment or checkups. To make sure your child's care is appropriate
for the stage of disease, work closely with the medical team. Learn as much as
you can about your child's disease and treatments, and stay on schedule with
medicine and exercise.
Because pain, stiffness, and swelling can
change from day to day, be sure to learn how to assess your child's
condition. It can be hard to know if children are having pain. Some children
are not able to say what they feel, while others are afraid to say they feel
pain if they think they will have to go to the doctor or think they will make
their parents upset. Children also simply learn to cope with pain by sleeping
or playing. To know a child is in pain, you may need to look for changes such
as stiff movements, rubbing a joint or muscle, or avoiding movement. You may also notice your child is irritable or easily
upset.
Initial treatment
Treatment for
juvenile idiopathic arthritis (JIA) usually begins
after your doctor has eliminated other causes for your child's symptoms. A good
indicator of JIA is if your child's pain, swelling, and stiffness in the joints
have persisted for at least 6 weeks. Your doctor may set up a treatment team,
often including a pediatrician, rheumatologist, and physical and/or
occupational therapist.
Physical exercise is a crucial part of
treatment for a child with JIA. Your child's
physical and
occupational therapists can teach you and your child
exercises to do at home to prevent
contractures and maintain joint range and muscle
strength. Moving your child's arthritic joints regularly through their full
range of motion helps prevent stiffening or deformity.
Many children with JIA don't want to move painful joints and need to be
encouraged to continue with daily physical therapy.
Medicine will
likely be an important factor in your child's treatment.
- Unless your child's condition is
life-threatening or involves severe eye or joint inflammation, nonsteroidal
anti-inflammatory drugs (NSAIDs) are likely to be the first line
of medication treatment to reduce inflammation and any pain. If you see no
improvement after 6 weeks, your doctor may try a different NSAID. Some children
gain relief from one NSAID but not another.
- In cases of severe
JIA, your doctor may prescribe medicines referred to as
disease-modifying antirheumatic drugs (DMARDs). (DMARDs are sometimes called slow-acting antirheumatic drugs, or SAARDs.) Methotrexate, either alone or in combination with other medicines, is the DMARD that is usually tried first. A type of DMARD called a biologic, such as etanercept (Enbrel), may also be tried.
- A corticosteroid injection into a joint also may
be used to reduce inflammation, particularly if your child has
oligoarticular JIA (formerly known as pauciarticular JIA).
Pain relief techniques can help you and your child
control pain caused by JIA. Your child's doctor can work with you to set up a
pain management plan, which might include heat treatments, exercise, and
cognitive-behavioral therapy.
Breathing and relaxation techniques can be an
effective way to reduce pain intensity.
Inflammatory eye disease may develop in children with
JIA. Because this form of eye disease generally has no symptoms and can lead to
a permanent decrease in vision or blindness, part of your child's treatment
plan should be regular checkups with an
ophthalmologist. Most children who develop eye disease
are treated with corticosteroids and prescription eyedrops called
mydriatics.
Home treatment to
help your child function as normally as possible should include and address
activities in the home, school, and community.
-
Range-of-motion exercises, done twice
daily with the assistance of an adult, will help to maintain joint range and
muscle strength and prevent contractures.
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Balancing rest and activity may mean extra naps or quiet times during the day, mixed with
frequent activity to keep muscles from stiffening and
weakening.
- Assistive devices can help your child hold onto, open,
close, move, or do things more easily. Doorknob extenders, Velcro fasteners,
and canes are all assistive devices.
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Partnering with school staff to develop creative ways of dealing with JIA-caused limitations
can help your child make the best of his or her abilities.
Ongoing treatment
After your child's initial
treatment for
juvenile idiopathic arthritis (JIA), he or she will likely require ongoing treatment throughout childhood. Many children
who have JIA will outgrow their disease and lead normal adult lives, while others
will have some disability and will need continued treatment as adults. Physical
exercise and medicines will be the basics of treatment throughout the disease's
course.
Physical therapy is a vital component of the
successful ongoing management of JIA. Help your child understand the importance
of physical therapy exercises and help him or her keep an upbeat attitude about
twice-or-more daily stretching and strengthening sessions. Working closely with
a pediatric physical therapist can be especially helpful.
Occupational therapy is often recommended. An occupational therapist (OT) can help a child learn ways to do self-care activities, play, and participate in school without making their symptoms worse.
If your
child doesn't respond to
NSAID treatment (first-line treatment) after 2 or 3
months, additional medicine (second-line treatment) will be necessary to manage
symptoms and inflammation. Methotrexate has been found to be the most effective
second-line medicine for children with JIA.6 Children
who don't respond well to methotrexate can be offered similar medicines,
sometimes referred to as
disease-modifying antirheumatic drugs (DMARDs) or a type of DMARD called a biologic.
Inflammatory eye disease can develop as a complication in children with JIA. Regular eye
examinations with an
ophthalmologist need to be included in your child's
treatment plan. Most children who develop eye disease are treated with
corticosteroids and prescription eyedrops called mydriatics. More severe or
continuing eye disease may require other medicines such as
methotrexate.
Treatment if the condition gets worse
If your
child develops a severe type of
juvenile idiopathic arthritis (JIA), your child's
treatment team will initiate treatments for more aggressive disease.
Physical therapy will be an important part of
treatment if your child is experiencing severe JIA. Regular physical exercise
will help maintain joint range and muscle strength and prevent
contractures. If your child is 4 years old or younger,
an adult will need to move the child's joints through the
range-of-motion exercises. Range-of-motion exercises
may be painful during a flare-up of arthritis, so it is very important to be
gentle. The
physical therapist can help set up an exercise program
for your child, either for the child to do alone or to do with help from an
adult. Exercises should be done every day and periodically reviewed by the
physical therapist.6 The therapist will be sure the
exercises are being done correctly and decide whether any exercises should be
added, dropped, or changed.
Occupational therapy will also be important. Working with an occupational therapist, a child learns to be as independent as possible, and find ways to play and participate in school activities.
Combination therapy—using
methotrexate with other medicines such as sulfasalazine, hydroxychloroquine, or
etanercept—may be used to treat children with severe JIA.
Biologic therapy (also called biological therapy) is a new option for treating JIA, particularly
polyarticular JIA, that does not respond to other
treatments. The biologic agent
etanercept has had some success in relieving symptoms and decreasing the number
of flare-ups. Other biologics, such as infliximab, are also used.
Surgery may be used in a very small number of
children with JIA who have severe joint deformity, loss of movement, or pain.
Inflammatory eye disease can develop as a complication
in children with JIA. Regular eye examinations with an
ophthalmologist need to be included in your child's
treatment plan. Most children who develop eye disease are treated with
corticosteroids and prescription eyedrops called mydriatics. More severe or
continuing eye disease may require other medicines such as methotrexate. If eye
disease does not respond to these treatments either, cyclosporine or biologics such as etanercept may help.6
What To Think About
Some children with JIA suffer a
loss of appetite severe enough that malnutrition becomes a medical concern. If
your child has little appetite for food, consult a nutritionist for help with
your child's basic nutritional needs.
Very few children with JIA have joint damage that
requires surgery. If at all possible, joint reconstruction is delayed until
childhood bone growth is complete (about 18 years of age).
Prevention
Currently, the cause of
juvenile idiopathic arthritis (JIA) is not well
understood, and there is no way to prevent it. The self-care methods listed
below may help prevent complications and make managing the illness
easier.
Preventing joint pain and swelling
Children who have
JIA need a careful balance of activity and rest. Encourage your child not to
overdo activity when he or she is feeling well. Too much activity will
generally make soreness worse. Limit your child's participation in activities
that are stressful to joints (such as running or contact sports) during flares
of arthritis. But try not to discourage activity so that the child begins to
feel very different from his or her playmates or friends.
Be sure
that your child takes his or her medicine as prescribed. Use joint supports or
splints if your doctor recommends them. Apply heat to stiff and painful joints
for 20 minutes, repeating as needed. You can use hot water bottles, heating
pads on a low-to-medium setting, or hot packs, either towels dipped in warm
water or wet towels microwaved for 15 to 30 seconds. Do not leave a small child
unattended with a heating pad. Always make sure heating pads, hot water
bottles, and hot packs are not too hot for your child's skin.
Preventing morning stiffness
Many children who have
JIA have less stiffness in the morning if their joints are kept warm during the
night. Footed pajamas or thermal underwear, or a sleeping bag, heated water
bed, or electric blanket may help keep joints warm.
Encourage your
child to take a warm bath or shower first thing in the morning to help ease
stiffness and then to stretch gently afterward.
Give morning
medicines as early as possible, with a snack or breakfast to prevent upsetting
an empty stomach.
Home Treatment
Living with
juvenile idiopathic arthritis (JIA), a childhood
disease that causes inflamed, swollen joints, often means making lifestyle
changes and adjustments. This can be frustrating and demanding for you, your
child, and your family. But many children with JIA do not have long-term
disease and disability and go on to lead healthy adult lives. To help both you
and your child cope with the challenges of chronic illness, work as a team with
your child's doctors and other health professionals.
Home, school, and community activities
Regular
exercise, taking medicines, and using assistive devices when needed will help
your child function as normally as possible at home and school.
Range-of-motion exercises. Children with juvenile
idiopathic arthritis (JIA) must do regular exercises to maintain joint range
and muscle strength and prevent
contractures. If you have an infant or child younger
than 4 years of age who has arthritis, an adult will need to move the child's
joints through the range-of-motion exercises. Older children can do the
exercises themselves but may still need adult supervision. Participation in
activities such as swimming or biking with other children helps improve a
child's ability to function, builds self-confidence, and may decrease pain and
disability.
Balancing rest and activity. Children
with JIA may need extra naps or quiet time during the day to rest their joints
and regain their strength. But long periods without activity can cause your
child's joints to be less flexible and may eventually lead to weakness in
unused muscles. It is also important not to overdo activity, particularly if it
causes pain or stiffness the following day.
Taking medicines. Sticking to a medication schedule can be difficult for
children with JIA. An older child may find it easier to remember to take
medicine by using a pillbox or chart for a day's or week's worth of medicine.
Ask your doctor whether the dose of medicine can be adjusted so your child can
take it at times that are most convenient and will not make him or her feel
"different." To avoid stomach upset, you can also give nonsteroidal
anti-inflammatory drugs (NSAIDs) with meals or a small snack.
Assistive devices. Items that can help
your child hold onto, open, close, move, or do things more easily include:
- Doorknob extenders, to avoid twisting the
wrist to open doors.
- Extended or enlarged handles on keys, pencils,
silverware, combs, or toothbrushes that make it easier to hold and use these
objects.
- Lightweight clothing and toys.
- Velcro
fasteners or simple, large fasteners on clothing, instead of small buttons or
snaps.
- A large pull tab or a loop of cord on a zipper, to make
zipping clothing easier.
- Elevated toilet seats, to avoid bending.
- Canes or crutches, to assist walking.
Addressing school issues. Your child's
teachers, school nurse, cafeteria staff, and physical education teachers can
become helpful partners as your child copes with JIA at school. Work with them
to develop creative ways of dealing with your child's limitations and making
the best of his or her abilities. If your child has trouble walking distances,
see whether your child's classes can be scheduled to minimize walking and stair
climbing. If your child gets stiff sitting still during class, perhaps the
teacher can encourage him or her to wiggle around and stretch during the class.
If your child has trouble writing neatly, he or she might try using a larger
pencil or pen. Ask your child's physical or occupational therapist for other
ideas. Be sure to learn about your child's rights under the Individuals with
Disabilities Education Act (IDEA) and other federal and state laws regarding
the education of children with disabilities.
Inflammatory eye disease can develop as
a complication in children with JIA. Make sure your child has regular eye
examinations with an
ophthalmologist. The eye disease associated with JIA
often has no symptoms, although blurred vision may be an early symptom.
Children with oligoarticular JIA need the most frequent examinations, but children with RF-negative polyarticular, enthesitis-related, and psoriatic arthritis also have an increased risk of eye problems. Talk to your doctor about how often your child should have an eye exam.
Juvenile idiopathic arthritis is a serious disease, but a better understanding of the forms of the disease, early treatment, and better medicines are all helping to improve the long-term outlook. The outlook
is even better when you and your child actively manage your child's health.
With greater understanding of the disease, you and your child will have less
fear, make better decisions, and have better results.
Take good physical care of yourself so that you can help your child
through the more difficult periods of illness. Consider becoming involved with
a support group of families who live with juvenile idiopathic arthritis. Your
local chapter of the Arthritis Foundation can provide classes and support group
information.
Medications
Most children with
juvenile idiopathic arthritis (JIA) need to take
medicine to reduce inflammation and control pain and to help prevent increasing
damage to the joints. When inflammation and pain are controlled, a child is
more willing and able to do joint exercises to improve joint strength and
prevent loss of movement.
Many different medicines are used to
treat JIA. No single medicine works for every child. It may take some time to
find the right medicine or combination of medicines that best controls your
child's symptoms. Treatment is tailored to each child by his or her
doctor and parents while considering effectiveness, side effects, cost, and the
type and severity of the disease.
Medication Choices
Although treatment varies depending on the needs of the
individual child, certain medications are often tried first (first-line
medications), while others are often saved to try later if they are needed
(second-line medications).
First-line medication. Nonsteroidal anti-inflammatory drugs (NSAIDs) are usually the first medicines tried to
control JIA inflammation and symptoms. Naproxen is the most frequently
used NSAID treatment for JIA. Doctors choose naproxen based on its low
incidence of side effects compared to its effectiveness.6 Ibuprofen is an effective alternative. But in general, less
than one-third of children will have significant relief from NSAIDs.3
Corticosteroids may also be used as a first-line
medication, especially as injections, for children who have
just a few joints affected or who have enthesitis. Oral or
intravenous (IV) corticosteroids are often used for
widespread joint pain or systemic problems such as fever or pericarditis. Corticosteroids work faster than some other drugs, so they may also be used until other medicines start working.
Second-line medication. If symptoms are not
well-controlled with NSAIDs or corticosteroids, stronger medicines such as
methotrexate are often used successfully.7
Methotrexate, sulfasalazine, and other second-line medicines are sometimes
referred to as
disease-modifying antirheumatic drugs (DMARDs). Some
experts prefer to call them slow-acting antirheumatic drugs (SAARDs).
Some children with JIA gain significant benefit from early methotrexate
treatment. Although there is no definitive way of knowing which children are
the best candidates for early methotrexate treatment, this practice is becoming
more common in an effort to prevent joint and eye damage. Early treatment with
methotrexate is often used for polyarticular JIA.3
Biologic therapy is a newer option to treat
JIA that does not respond to other treatments.
Biologics such as etanercept have had some success in relieving symptoms and decreasing the number of flare-ups.
Medications used to treat JIA
First-line
-
Nonsteroidal anti-inflammatory drugs (NSAIDs)
-
Corticosteroids
Second-line
-
Corticosteroids
-
Methotrexate
-
Biologics such as etanercept (Enbrel),
infliximab (Remicade), adalimumab (Humira), and abatacept (Orencia)
Other second-line medications used less often
-
Sulfasalazine
-
Antimalarials (such as hydroxychloroquine sulfate
[Plaquenil])
-
Adult therapies, such as cytotoxic (cell-destroying)
drugs and intravenous human immunoglobulin, that may be used for rheumatoid
arthritis in adults but are not yet proved to be safe and effective for
children with JIA
Gold salts were one of the first treatments used for
joint inflammation, and you may still hear about them. But injected gold salts
have been replaced by methotrexate for the treatment of JIA. Gold salts taken
by mouth (oral) have not been shown to be effective for JIA.6
Medications used to treat inflammatory eye disease
-
Corticosteroid
eyedrops
-
Methotrexate and cyclosporine A
-
Mydriatics
, which are eyedrops that dilate the pupil
and keep the iris from sticking to the cornea or lens
-
Biologics
What To Think About
Annual flu shots are recommended
for children who are on long-term aspirin therapy. Children on long-term
aspirin therapy who get
chickenpox or
influenza (flu) are at risk for getting Reye
syndrome. Although there is a risk, Reye syndrome is very rare. Very few
cases of Reye syndrome have been reported in children with chronic arthritis
who were being treated with aspirin. If your child has been exposed to
chickenpox or flu, talk to the doctor about giving your child acetaminophen to
control pain and relieve fever until the incubation period, or the illness
itself, has passed.
Combination therapy—such as using methotrexate
with sulfasalazine, hydroxychloroquine, or etanercept—has been used on a
limited basis to treat JIA. Most medical experience with combination therapy is
with adults. Only children with severe JIA that has not improved with
methotrexate or sulfasalazine are considered for combination treatment.
It is impossible to predict whether a child will improve with a certain
medicine. Several different medicines may be tried before one is found that
controls symptoms and doesn't cause side effects. It can also take weeks to
months for a medicine to show effect, and symptoms may continue during that
time.
Surgery
Surgical treatment may be used in a very small
number of children who have
juvenile idiopathic arthritis (JIA) who have severe
joint deformity, loss of movement, or pain. Surgery is a possible treatment
option if your child has not improved with medicine and physical therapy and is
unable to walk or perform manual tasks.
Surgery Choices
When surgery to correct joint deformity is needed, the
more commonly used procedures include:
-
Soft tissue releases of contractures,
which involve cutting the muscles attached to an abnormally bent joint. As the
muscles and other shortened tissues are released, the affected joint can return
to a more normal position.
-
Total joint replacement, which may be considered as a last resort for joints that
have been so badly damaged by JIA that walking is very difficult or impossible.
Important considerations for you to think about
include your child's age, the number of joints involved in the disease, and the
impact on your child's mobility.
Other surgical procedures that have been used in children
who have JIA but are recommended only in selected cases include:
-
Osteotomy, which
involves removing a wedge of bone to allow more normal alignment of the joint.
An osteotomy may be recommended for children who have severe joint
contractures.
-
Epiphysiodesis
,
in which the portion in a long leg bone where growth occurs is removed in order
to stop growth.
-
Synovectomy or tenosynovectomy, rarely used for JIA. Synovectomy involves the surgical
removal of the joint lining (synovium) and/or the covering of the tendon
(tenosynovectomy) to reduce joint inflammation.
-
Arthrodesis
, rarely used in children, which involves
the fusion of two bones in a diseased joint so that the joint can no longer
move.
What To Think About
The main things to think about
for surgery during childhood are the child's age and whether his or her bones
are still growing. When considering total joint replacement, it is also
important to consider the possibility of needing another joint replacement in
10 to 20 years. The timing often requires a balance between the child's age,
the expected life of the replaced joint, and the possible loss of bone and
muscle strength if surgery is delayed too long.
Other Treatment
Physical and
occupational therapy are vital to the successful
management of
juvenile idiopathic arthritis (JIA). Maintaining good
joint function and range of motion and being able to do daily tasks help a
child who has JIA develop normally.
Other Treatment Choices
Physical and occupational therapy
The purpose of
physical therapy is to decrease pain and increase strength and range of motion,
to allow your child to resume or continue normal activities. Occupational
therapy works to help a child live as independently as possible.
- Physical conditioning may include aerobic
exercise, range-of-motion exercises, and strength and stretching
exercises.
-
Stretching and strengthening exercises can help a child maintain strength and a normal
range of motion.
-
Splinting at night will help keep the
wrist, hand, knee, and/or ankle joints straight, which may prevent pain,
morning stiffness, and contractures. Working splints can help support a joint
and relieve pain when writing or doing other hand tasks.
-
Serial casting of the knees, ankles, wrists, fingers, and/or elbows is a
temporary straightening and casting of the affected joint. The cast is then
removed, the child goes through some physical therapy, and a new cast is
applied with the joint stretched a bit more.
-
Shoe lifts or inserts help to equalize leg lengths for children in whom one
leg grows at a different rate than the other.
Nutrition
Healthy eating means eating a variety
of foods so that your child gets the nutrients he or she needs for growth and
development. Good nutrition will also help fight the effects of JIA. Important
nutrients include protein, carbohydrate, fat, vitamins, and minerals. Your
child can eat all types of food as long as his or her weekly intake is balanced
and varied.
- As part of a healthy diet for a child with
JIA, your child's doctor may recommend
vitamin D and calcium. These nutrients can help
control bone loss that is often linked with inactivity and with corticosteroid
treatment.
Some nutrients are thought to help reduce inflammation,
so they may help decrease some symptoms of JIA.
- Vitamin C is an antioxidant that may help
reduce inflammation in the body. Vitamin C is found in citrus fruits, tomatoes,
berries, broccoli, cabbage, and brussels sprouts.
- Omega-3 fatty
acids in fish oil have been shown to mildly reduce inflammation in adults with
rheumatoid arthritis and may have the same effect in children who have JIA. The
best sources of omega-3 fatty acids are cold-water fish and flaxseed
oil.8
Pain management
Complementary medicine therapies for pain management
-
Massage is used to promote relaxation,
relieve pain, and restore normal joint movement.
-
Guided imagery may be used to promote relaxation and manage
pain.
-
Acupuncture is mildly effective in relieving pain in
adults who have rheumatoid arthritis and may help relieve pain in children who
have JIA.
Other Places To Get Help
Organizations
|
American Academy of Orthopaedic Surgeons
(AAOS)
|
| 6300 North River Road |
| Rosemont, IL 60018-4262 |
| Phone: |
1-800-346-AAOS (1-800-346-2267)
(847) 823-7186 |
| Fax: |
(847) 823-8125 |
| Email: |
pemr@aaos.org |
| Web Address: |
www.aaos.org |
| |
|
The American Academy of Orthopaedic Surgeons (AAOS) provides
information and education to raise the public's awareness of musculoskeletal
conditions, with an emphasis on preventive measures. The AAOS Web site contains
information on orthopedic conditions and treatments, injury prevention, and
wellness and exercise.
|
|
|
Juvenile Arthritis Alliance (JA
Alliance)
|
| P.O. Box 7669 |
| Atlanta, GA 30357-0669 |
| Phone: |
1-800-283-7800 |
| Web Address: |
www.arthritis.org/ja-alliance-main.php |
| |
|
The Juvenile Arthritis Alliance (JA Alliance or JAA) is
a virtual community connected through the Arthritis Foundation Web site. Its
members are parents, volunteers, health professionals, and anyone who is
affected by juvenile arthritis. The JAA works to reach children and families
with developmentally appropriate quality programs and services. Such programs
and services include national and regional JA conferences, juvenile arthritis
camps, evidence-based JA programs, and chapter level informational workshops
(family days that focus on children and youth newly diagnosed with arthritis
and on their families). The JAA Web site includes information on juvenile
arthritis issues such as treatment and dealing with juvenile arthritis in
everyday life. The Web site also offers information on networking, advocacy,
and research.
|
|
|
KidsHealth for Parents, Children, and
Teens
|
| 10140 Centurion Parkway North |
| Jacksonville, FL 32256 |
| Phone: |
(904) 697-4100 |
| Fax: |
(904) 697-4125 |
| Web Address: |
www.kidshealth.org |
| |
|
This Web site is sponsored by the Nemours Foundation. It
has a wide range of information about children's health, from allergies and
diseases to normal growth and development (birth to adolescence). This Web site
offers separate areas for kids, teens, and parents, each providing
age-appropriate information that the child or parent can understand. You can
sign up to get weekly e-mails about your area of interest.
|
|
|
National Institute of Arthritis and Musculoskeletal and
Skin Diseases (NIAMS), National Institutes of Health
|
| 1 AMS Circle |
| Bethesda, MD 20892-3675 |
| Phone: |
1-877-22-NIAMS (1-877-226-4267) toll-free
(301) 495-4484 |
| Fax: |
(301) 718-6366 |
| TDD: |
(301) 565-2966 |
| Email: |
niamsinfo@mail.nih.gov |
| Web Address: |
www.niams.nih.gov |
| |
|
The National Institute of Arthritis and Musculoskeletal
and Skin Diseases (NIAMS) is a governmental institute that serves the public
and health professionals by providing information, locating other information
sources, and participating in a national federal database of health
information. NIAMS supports research into the causes, treatment, and prevention
of arthritis and musculoskeletal and skin diseases and supports the training of
scientists to carry out this research.
The NIAMS Web site provides
health information referrals to the NIAMS Clearinghouse, which has information
packages about diseases.
|
|
|
Spondylitis Association of America
|
| P.O. Box 5872 |
| Sherman Oaks, CA 91413 |
| Phone: |
1-800-777-8189
(818) 981-1616 |
| Email: |
info@spondylitis.org |
| Web Address: |
www.spondylitis.org |
| |
|
The Spondylitis Association of America (SAA) is a national
nonprofit organization. It is dedicated to the cure of ankylosing spondylitis and
related diseases such as spondyloarthropathy, psoriatic arthritis, reactive arthritis, and enteropathic arthritis through education, advocacy, awareness, and research.
|
|
References
Citations
-
Anthony KK, Schanberg LE (2003). Pain in children with
arthritis: A review of the current literature. Arthritis and Rheumatism, 49(2): 272–279.
-
Labyak SE, et al. (2003). Sleep quality in children
with juvenile rheumatoid arthritis. Holistic Nursing Practice, 17(4): 193–200.
-
Hashkes PJ, Laxer RM (2005). Medical treatment of
juvenile ideopathic arthritis. JAMA, 294(13):
1671–1684.
-
Nistala K, et al. (2009). Juvenile idiopathic arthritis. In
GS Firestein et al., eds., Kelley's Textbook of Rheumatology, 8th ed., vol. 2, pp. 1657–1675. Philadelphia: Saunders
Elsevier.
-
Warren RW, et al. (2005). Juvenile idiopathic
arthritis (Juvenile rheumatoid arthritis). In WJ Koopman, LW Moreland, eds.,
Arthritis and Allied Conditions, 15th ed., vol. 1, pp.
1277–1300. Philadelphia: Lippincott Williams and Wilkins.
-
Giannini EH, Brunner HI (2005). Treatment of juvenile
rheumatoid arthritis. In WJ Koopman, LW Moreland, eds., Arthritis and Allied Conditions, 15th ed., vol. 1, pp.
1301–1318. Philadelphia: Lippincott Williams and Wilkins.
-
Miller ML, Cassidy JT (2007). Juvenile rheumatoid
arthritis. In RM Kliegman et al., eds., Nelson Textbook of Pediatrics, 18th ed., chap. 154, pp. 1001–1011. Philadelphia:
Saunders.
-
Murray MT, Pizzorno JE Jr (2006). Rheumatoid
arthritis. In JE Pizzorno, MT Murray, eds., Textbook of Natural Medicine, 3rd ed., vol. 2, pp. 2089–2108. St. Louis:
Churchill Livingstone Elsevier.
Other Works Consulted
- Duffy CM, et al. (2005). Nomenclature and
classification in chronic childhood arthritis. Arthritis and Rheumatism, 52(2): 382–385.
- Goldmuntz EA, White PH (2006). Juvenile idiopathic arthritis: A review for pediatricians. Pediatrics in Review, 27(4): e24–e32.
- Simon L, et al. (2002). Treatment of pain in children
and older adults with arthritis. In Guideline for the Management of Pain in Osteoarthritis, Rheumatoid Arthritis, and Juvenile Chronic Arthritis, 2nd ed., chap. 5, pp. 119–129. Glenview, IL: American
Pain Society.
- Wilson D, et al. (2007). Juvenile rheumatoid arthritis
(juvenile idiopathic arthritis) section of The child with musculoskeletal or
articular dysfunction. In Wong's Nursing Care of Infants and Children, 8th ed., chap. 39, pp. 1791–1798. St. Louis: Mosby
Elsevier.
Credits
|
By
|
Healthwise Staff |
|
Primary Medical Reviewer
|
John Pope, MD - Pediatrics |
|
Specialist Medical Reviewer
|
Stanford M. Shoor, MD - Rheumatology |
|
Last Revised
|
June 11, 2010 |