Juvenile Rheumatoid Arthritis

Topic Overview

What is juvenile rheumatoid arthritis?

Juvenile rheumatoid arthritis is a childhood disease that causes inflamed, swollen joints . This makes joints stiff and painful.

Unlike adults with rheumatoid arthritis, many children with the disease grow out of it after they get treatment. Others will need ongoing treatment as adults.

There are three types of juvenile rheumatoid arthritis.

• Pauciarticular is the most common and mildest type. Your child may have pain in 1 to 4 joints, such as the knees, ankles, fingers, toes, wrists, elbows, or hips.
• Polyarticular is more severe. It affects more joints and tends to get worse over time.
• Systemic is the least common type. But it can be the most serious. It causes pain in many joints and can also spread to organs.

What causes juvenile rheumatoid 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.

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 rheumatoid 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. 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 rheumatoid 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 rheumatoid 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 with 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

Cause

The cause of juvenile rheumatoid arthritis (JRA) 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 JRA reported a higher occurrence of other autoimmune diseases in these families. It is possible that these families share genes that make them more susceptible to autoimmune diseases, including JRA.¹

An increasing number of international experts are now referring to JRA 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 rheumatoid arthritis (JRA) 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 JRA, 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.² 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 JRA a child has.

Eye disease usually causes no symptoms before permanent vision loss occurs. For this reason, it is very important for a child with JRA 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 JRA 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 JRA 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 similar symptoms to JRA 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. JRA is a relatively uncommon cause of these symptoms.

Some children have forms of chronic arthritis that are similar to yet distinct from juvenile rheumatoid 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 rheumatoid arthritis (JRA) is unpredictable, especially during the first few years after a child is diagnosed. JRA, also called juvenile idiopathic arthritis (JIA) 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 JRA 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.³ Most children have good and bad days.

Of all children with JRA, 3 or 4 out of 10 children will have long-term disability.⁴ While the overall long-term outlook for children with JRA 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 JRA 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 rheumatoid arthritis he or she has. While a child with pauciarticular JRA (4 or fewer joints affected) has a good long-term outlook other than eye disease risk, a child with polyarticular JRA (5 or more joints) or systemic JRA (whole-body symptoms) is likely to have more long-term problems.⁵

Pauciarticular JRA (oligoarthritis)

About 40% to 60% of all children affected by JRA have the pauciarticular form.⁴ Some children with more severe disease have joint damage that shows on X-rays within 5 years. Children with pauciarticular JRA (oligoarthritis, meaning "few joints") have a 40% to 50% chance of continuing to have disease as an adult.⁴ Among children with pauciarticular JRA:⁵

• Most children have 4 or fewer joints affected.
• Some go on to have 5 or more joints affected over time (referred to as 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.⁴

Polyarticular JRA (polyarthritis)

Polyarticular JRA (polyarthritis) affects many joints-often the knee, hip, wrist, elbow, and ankle joints-and may affect the small joints in the hands and feet. This type of JRA is more severe than pauciarticular JRA 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 25% to 35% of children affected by JRA have the polyarticular form. Of these children, about 50% to 70% will have active disease that continues into adulthood.⁴

For a child with polyarticular JRA, the following are signs of increased risk of developing joint deformity and disability as an adult:⁵

• Onset of disease later in childhood
• Joint problems in hands or feet early in the course of the disease
• Rapidly progressing joint damage
• Chronic symptoms that don't respond to treatment
• Significant whole-body (systemic) symptoms, such as fever, fatigue, and appetite loss
• Bumps under the skin (rheumatoid nodules) over pressure points (such as the elbow or back of the heel)

Of all children with juvenile rheumatoid arthritis, only about 5% to 10% have polyarticular JRA with the presence of the rheumatoid factor (RF) antibody in their blood.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 JRA 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 JRA.⁶

Systemic JRA

About 10% to 20% of children affected by JRA have the systemic form.⁴ Usually, a child with systemic JRA 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. About a third of children with systemic JRA develop heart and complications.⁶

Some children with systemic JRA will have joint damage visible on X-ray within 2 years. About 50% to 70% of children with systemic JRA will continue to have active disease as adults.⁴ Systemic symptoms rarely last more than 5 years, while joint symptoms can last 10 years or more.⁵

Complications

Complications associated with JRA 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 JRA and can occur early. About 30% of children with JRA will have some level of disability that continues into adulthood.⁷ Children with the pauciarticular form of JRA seldom develop significant disabilities.

Some children with polyarthritis develop arthritis in the neck that can cause the neck bones to fuse together. A child who has arthritis in the neck may need to wear a neck (cervical) collar when riding in a vehicle to reduce the risk of neck injury during a sudden stop or accident.

Complications of systemic JRA 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 rheumatoid arthritis (JRA) are known at this time. But a recent study of relatives of children with JRA reported a higher occurrence of other autoimmune diseases in these families. It is possible that these families share genes that make them more susceptible to autoimmune diseases, including JRA.¹

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 indicate an infection in the joint.

Who To See

For initial evaluation of joint pain and other symptoms of juvenile rheumatoid arthritis (JRA), consult with a:

• Family medicine doctor.
• Pediatrician.
• Nurse practitioner (NP).
• Physician assistant (PA).

For additional testing and disease management, consult with a rheumatologist who specializes in children's rheumatic disease (pediatric rheumatologist).

The disease management team for JRA 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 rheumatoid arthritis (JRA). In most cases, routine lab results do not point to an obvious diagnosis of this disease. JRA 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:

• Medical history and physical examination
• Complete blood count (CBC)
• Erythrocyte sedimentation rate (ESR, or sed rate)
• Urinalysis
• Rapid strep test or throat culture (to test for strep throat)

The following tests are done if needed:

• Rheumatoid factor (RF), to clarify whether a child with polyarticular JRA is RF-positive or RF-negative
• Antinuclear antibody (ANA), to clarify a child's type of JRA and risk for eye disease (a positive ANA result is a marker for high eye disease risk, usually found in girls who develop pauciarticular JRA at a young age)
• X-ray
• Magnetic resonance imaging (MRI), which may reveal early joint damage
• Human lymphocyte antigen or HLA-B27 genetic test, which may help distinguish JRA from other types of arthritis that affect children, such as ankylosing spondylitis

Early Detection

There are no standard screening tests that are used to identify children who may develop juvenile rheumatoid arthritis (JRA).

Early eye disease detection

Slit lamp eye examinations are necessary for all children with juvenile rheumatoid 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 JRA 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 JRA symptoms are. In fact, children at greatest risk are girls who develop mild pauciarticular 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 rheumatoid arthritis (JRA) are to reduce your child's joint pain and to prevent disability. Physical therapy and medicine are the basis of medical treatment for JRA, also called juvenile idiopathic arthritis (JIA) or juvenile chronic arthritis.

Treatment is determined by the type and severity of JRA. Even when JRA is uncomplicated, an affected child still needs many years of medical treatment. 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, it is important 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.⁸ You may also notice your child is irritable or easily upset.

Initial treatment

Treatment for juvenile rheumatoid arthritis (JRA) usually begins after your doctor has eliminated other causes for your child's symptoms. A good indicator of JRA 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 JRA. 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 JRA 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 JRA, your doctor may prescribe medicines referred to as disease-modifying antirheumatic drugs (DMARDs) or slow-acting antirheumatic drugs (SAARDs). DMARDs/SAARDs that may be prescribed for JRA include methotrexate, either alone or in combination with other medicines, and/or a newer DMARD called etanercept (Enbrel), which is a tumor necrosis factor (TNF) inhibitor.
• A corticosteroid injection into a joint also may be used to reduce inflammation, particularly if your child has pauciarticular JRA (oligoarthritis).

Pain relief techniques can help you and your child control pain caused by JRA. 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.⁸

Inflammatory eye disease may develop in children with JRA. 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.⁹

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.
• 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.
• Partnering with school staff to develop creative ways of dealing with JRA-caused limitations can help your child make the best of his or her abilities.

Ongoing treatment

After your child's initial treatment for juvenile rheumatoid arthritis (JRA), it is likely that he or she will require ongoing treatment throughout childhood. Many children with JRA 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 JRA. 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.

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 JRA.⁹ Children who don't respond well to methotrexate can be offered similar medicines, sometimes referred to as disease-modifying antirheumatic drugs (DMARDs) or slow-acting antirheumatic drugs (SAARDs).

Inflammatory eye disease can develop as a complication in children with JRA. 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.⁹

Treatment if the condition gets worse

If your child develops a severe type of juvenile rheumatoid arthritis (JRA), 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 JRA. 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.⁹ The therapist will be sure the exercises are being done correctly and decide whether any exercises should be added, dropped, or changed.

Combination therapy-using methotrexate with other medicines such as sulfasalazine, hydroxychloroquine, or etanercept-may be used to treat children with severe JRA.

Biological therapy is a new option to treat JRA, particularly polyarticular JRA, that does not respond to other treatments. The biological agent etanercept, which is a tumor necrosis factor (TNF) inhibitor, has had some success in relieving symptoms and decreasing the number of flare-ups. Other TNF inhibitors, such as infliximab, are also used.¹⁰

Surgery may be used in a very small number of children with JRA who have severe joint deformity, loss of movement, or pain.

Inflammatory eye disease can develop as a complication in children with JRA. 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 TNF inhibitors such as etanercept may help.⁹

What To Think About

Some children with JRA 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.

Most children with JRA do not go on to have adult rheumatoid arthritis or other long-term problems related to JRA. Children with pauciarticular JRA (4 or fewer affected joints) have a good long-term outlook but an ongoing risk of developing eye disease. Children with polyarticular JRA (5 or more joints) tend to have more problems long-term.

Very few children with JRA 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).⁵

Prevention

Currently, the cause of juvenile rheumatoid arthritis (JRA) 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 with JRA 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 with JRA 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 rheumatoid arthritis (JRA), 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 most children with JRA 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 rheumatoid arthritis (JRA) 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 JRA 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 JRA. 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 JRA 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 JRA. Make sure your child has regular eye examinations with an ophthalmologist. The eye disease associated with JRA often has no symptoms, although blurred vision may be an early symptom. Children with disease in up to 4 joints (pauciarticular JRA) need the most frequent examinations.

Overall, juvenile rheumatoid arthritis (JRA) has a good 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 rheumatoid arthritis. Your local chapter of the Arthritis Foundation can provide classes and support group information.

Medications

Most children with juvenile rheumatoid arthritis (JRA) 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 JRA. 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 individualized for 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 JRA inflammation and symptoms. Naproxen sodium is the most frequently used NSAID treatment for JRA. Doctors choose naproxen based on its low incidence of side effects compared to its effectiveness.⁹ Ibuprofen is an effective alternative. But in general, less than one-third of children will have significant relief from NSAIDs.⁴

Corticosteroids may also be used as a first-line medicine, especially as injections, for children who have pauciarticular (oligoarthritis) with shortening of the muscles around the joints (contractures). Oral or injected corticosteroids are often used for polyarticular disease with joint pain and swelling, and sometimes intravenous (IV) corticosteroids are used for systemic JRA.¹¹

Second-line medication. If symptoms are not well-controlled with NSAIDs or corticosteroids, stronger medicines such as methotrexate are often used successfully.¹¹ Methotrexate, sulfasalazine, and other second-line medications are sometimes referred to as disease-modifying antirheumatic drugs (DMARDs). Some experts prefer to call them slow-acting antirheumatic drugs (SAARDs).

Some children with JRA 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 JRA.⁴

Biological therapy is a newer option to treat JRA (particularly polyarticular JRA) that does not respond to other treatments. The biological agent etanercept, which is a tumor necrosis factor (TNF) inhibitor, has had some success in relieving symptoms and decreasing the number of flare-ups. Other TNF inhibitors, such as infliximab, are also used.¹⁰

Medications used to treat JRA

First-line

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Corticosteroids

Second-line

Corticosteroids

Methotrexate

TNF inhibitors such as:

• Etanercept (Enbrel)
• Infliximab (Remicade)
• Adalimumab (Humira) and abatacept (Orencia), other medicines similar to etanercept and infliximab

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 JRA

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 JRA. Gold salts taken by mouth (oral) have not been shown to be effective for JRA.⁹

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

Tumor necrosis factor (TNF) inhibitors. These medicines are biological agents known as anti-TNF agents because they reduce inflammation by blocking the TNF protein. Etanercept is an example of these medicines.

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's syndrome. Although there is a risk, Reye's syndrome is very rare. Very few cases of Reye's 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 JRA. Most medical experience with combination therapy is with adults. Only children with severe JRA 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 with juvenile rheumatoid arthritis (JRA) 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 JRA 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 with JRA 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 JRA. 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 rheumatoid arthritis (JRA). Maintaining good joint function and range of motion and being able to do daily tasks help a child who has JRA 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.
• 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 JRA. 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 JRA, 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 JRA.

• 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 with JRA. The best sources of omega-3 fatty acids are cold-water fish and flaxseed oil.¹²

Pain management

• Breathing and relaxation techniques to relieve chronic pain include distraction, progressive muscle relaxation, and meditative breathing exercises.
• Heat, cold, or water therapy may reduce muscle spasms around joints, which can cause significant pain and movement problems.
• Stretching and strengthening exercises can help a child maintain strength and a normal range of motion.

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 JRA.

What To Think About

Physical therapy is a vital component of the successful management of juvenile rheumatoid arthritis. If possible (depending on age), help your child to understand the importance of physical therapy exercises and to keep an upbeat attitude about twice-or-more daily stretching and strengthening sessions. Working closely with a pediatric physical therapist can be especially helpful.

Other Places To Get Help

Organizations

References

Citations

1. Prahalad S, et al. (2002). Increased prevalence of familial autoimmunity in simplex and multiplex families with juvenile rheumatoid arthritis. Arthritis and Rheumatism, 46(7): 1851-1856.

2. Anthony KK, Schanberg LE (2003). Pain in children with arthritis: A review of the current literature. Arthritis and Rheumatism, 49(2): 272-279.

3. Labyak SE, et al. (2003). Sleep quality in children with juvenile rheumatoid arthritis. Holistic Nursing Practice, 17(4): 193-200.

4. Hashkes PJ, Laxer RM (2005). Medical treatment of juvenile ideopathic arthritis. JAMA, 294(13): 1671-1684.

5. Cassidy JT (2005). Juvenile rheumatoid arthritis. In ED Harris Jr et al., eds., Kelley's Textbook of Rheumatology, 7th ed., vol. 2, pp. 1579-1596. Philadelphia: Elsevier Saunders.

6. 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.

7. Wallace CA, Sherry DD (2003). Juvenile rheumatoid arthritis. In CD Rudolph et al., eds., Rudolph's Pediatrics, 21st ed., chap 12.4, pp. 836-840. New York: McGraw-Hill.

8. 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.

9. 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.

10. Hollister JR (2007). Rheumatic diseases. In WW Hay Jr et al., eds., Current Pediatric Diagnosis and Treatment, 18th ed., chap. 26, pp. 822-829. New York: McGraw-Hill.

11. 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.

12. 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

• Pisetsky DS, St Clair EW (2001). Progress in the treatment of rheumatoid arthritis. JAMA, 286(22): 2787-2790.

• Van der Linden S, van der Heijde D (1998). Ankylosing spondylitis: Clinical features. Rheumatic Disease Clinics of North America, 24(4): 663-676.

• 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.

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