Juvenile Idiopathic Arthritis

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Juvenile Idiopathic Arthritis

Topic Overview

Picture of the skeletal system What is juvenile idiopathic arthritis?

Juvenile idiopathic arthritis (JIA)—sometimes also called juvenile arthritis or juvenile rheumatoid arthritis—is a childhood disease that causes inflamed, swollen joints. This makes joints stiff and painful.

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 examinations 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 examination. 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 physiotherapy 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. Physiotherapists 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 physiotherapy.

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 counselling called cognitive-behavioural therapy. Breathing and relaxation exercises can also help ease your child’s pain.

Frequently Asked Questions

Learning about juvenile idiopathic arthritis:

Being diagnosed:

Getting treatment:

Ongoing concerns:

Living with juvenile idiopathic arthritis:

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 39.5°C (103°F) to 41°C (106°F), 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 [39.5°C (103°F) to 41°C (106°F)] 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 ASA or another non-steroidal 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 your family doctor, general practitioner, or your child's pediatrician. :

For additional testing and disease management, you may be referred to 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.

Examinations 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. Physiotherapy 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 physiotherapy.

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, non-steroidal 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-behavioural 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.
  • 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 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.

Physiotherapy is a vital component of the successful ongoing management of JIA. Help your child understand the importance of physiotherapy exercises and help him or her keep an upbeat attitude about twice-or-more daily stretching and strengthening sessions. Working closely with a pediatric physiotherapist 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.

Physiotherapy 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 physiotherapist 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 physiotherapist.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 non-steroidal 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 education rights. Provincial laws exist to provide services for disabled children, which includes those with JIA. In addition, there may be local laws or policies to aid children with JIA. Find out what services are available for your child in your area.

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 examination.

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. For more information, talk to your child's doctor or visit the Arthritis Society of Canada Web site at www.arthritis.ca.

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. Non-steroidal 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

Non-steroidal 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 ASA therapy. Children on long-term ASA 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 ASA. 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 physiotherapy 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 physiotherapy 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 physiotherapy, 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

Arthritis Society of Canada
393 University Avenue
Suite 1700
Toronto, ON  M5G 1E6
Phone: (416) 979-7228
Fax: (416) 979-8366
Email: info@arthritis.ca
Web Address: http://www.arthritis.ca/
 

The Arthritis Society provides funding for arthritis research and offers information on patient care, public education, and self-management of arthritis.


Canadian Arthritis Network
522 University Avenue
Suite 1002
Toronto, ON  M5G 1W7
Phone: (416) 586-4770
Fax: (416) 586-8395
Email: can@arthritisnetwork.ca
Web Address: www.arthritisnetwork.ca
 

The Canadian Arthritis Network (CAN) provides a variety of information about arthritis research and development in Canada.


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
4600 Touchton Road East, Building 200
Suite 500
Jacksonville, FL  32246
Phone: (904) 232-4100
Fax: (904) 232-4125
Web Address: www.kidshealth.org
 

This Web site is sponsored by 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.


U.S. 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 website provides health information referrals to the NIAMS Clearinghouse, which has information packages about diseases.


References

Citations

  1. Anthony KK, Schanberg LE (2003). Pain in children with arthritis: A review of the current literature. Arthritis and Rheumatism, 49(2): 272–279.
  2. Labyak SE, et al. (2003). Sleep quality in children with juvenile rheumatoid arthritis. Holistic Nursing Practice, 17(4): 193–200.
  3. Hashkes PJ, Laxer RM (2005). Medical treatment of juvenile ideopathic arthritis. JAMA, 294(13): 1671–1684.
  4. 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.
  5. 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.
  6. 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.
  7. 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.
  8. 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
Primary Medical Reviewer Andrew Swan, MD, CCFP, FCFP - Family Medicine
Specialist Medical Reviewer Stanford M. Shoor, MD - Rheumatology
Last Revised August 13, 2010

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information.