WebMD Encyclopedia Article: JRA

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Juvenile Rheumatoid Arthritis

http://my.webmd.com/encyclopedia/article/1834.50843Topic Overview

Juvenile rheumatoid arthritis (JRA) is a disease that affects children (age 16 or younger) and causes inflamed, swollen joints that are often stiff and painful.

There are 3 types of juvenile rheumatoid arthritis. Each type is based on the number of joints affected from the start. The 3 types are:

Pauciarticular JRA. This type of JRA affects 1 to 4 joints at the start of the disease. It is the most common type of JRA. Polyarticular JRA. This type of JRA affects more than 4 joints when it first starts. The child with this type of JRA is either RF-positive or RF-negative, depending on whether a certain antibody (called rheumatoid factor or RF) is present in his or her body. Systemic JRA. This type of JRA is the most serious but also the least common. The child with systemic JRA usually doesn't have pain in a specific joint at first but instead has whole-body symptoms, high fever spikes, generalized pain all over the body, and sometimes a rash.

Children with pauciarticular JRA can often be treated with mild, safe nonsteroidal anti-inflammatory drugs (NSAIDs); physical therapy; and occasional corticosteroid injections in a joint. For children with polyarticular JRA, NSAIDs, methotrexate and possibly low-dose corticosteroids or corticosteroid joint injections may be used. Few children need stronger drugs (disease-modifying antirheumatic drugs, or DMARDs), systemic corticosteroids, or surgery and most do not have lifelong problems related to juvenile rheumatoid arthritis.

Other forms of arthritis, called spondyloarthropathies and, less often, lupus or joint infection, can affect children. At times, it is difficult to tell the difference between these joint problems. For this reason, juvenile rheumatoid arthritis is often difficult to diagnose.

Inflammatory eye disease may develop as a complication in children with juvenile rheumatoid arthritis (JRA). It is especially common in girls who developed pauciarticular JRA and who have a positive antinuclear antibody (ANA) test result. All children with JRA need regular eye examinations by an ophthalmologist.

In Europe, juvenile rheumatoid arthritis and other forms of rheumatic disease in children are classified together as juvenile chronic arthritis (JCA). The diagnostic and inclusion criteria for the European classification (which is JCA) is somewhat different than the criteria used by the American College of Rheumatology (which is JRA). The difference in criteria results in differences in prevalence rates for Europe and America. See classification.

ClassificationCause

The cause of juvenile rheumatoid arthritis (JRA) is unknown. Most experts believe it may be caused by a combination of factors, including:

An overly-active immune response may cause the immune system to attack joint tissues by mistake, as if they were viruses or bacteria. Infection in the body, especially in systemic JRA. Some genetic factor that causes the person to be susceptible to getting the disease.

The following are not related to the cause of JRA:

Diet Climate Vitamin deficiency Injury Birth defects Disease during pregnancy

Who is affectedSymptoms

Some symptoms are common to all forms of juvenile rheumatoid arthritis (JRA), but each form also has unique symptoms.

Symptoms common to all 3 forms of juvenile rheumatoid arthritis (JRA) include:1, 2

Joint pain and swelling that comes and goes. Joint stiffness that is worse in the morning, at the end of the day, and after periods of inactivity (young children may be cranky, may not want to move, or may not want to use a particular joint). Often unpredictable changes in symptoms; from periods with no symptoms (remission) to severe flare-ups. Symptoms in children may be seen as irritability, refusal to walk, or protection or guarding of a joint. You might notice your child limping or avoiding the use of a certain joint.

Symptoms of pauciarticular JRA

Pauciarticular juvenile rheumatoid arthritis (1 to 4 joints affected) often affects only 1 large joint at first, most commonly the knee.

This type of JRA is common in the knees, ankles, and elbows and sometimes affects the jaw (temporomandibular joint), fingers, wrists, and neck. The hip is usually not affected. Symptoms are limited to joint swelling and pain and do not usually involve whole-body symptoms (systemic symptoms, such as weakness, tiredness, fever). Symptoms do not occur in the same joints on both sides of the body (asymmetric). Inflammation of the eye (iritis, iridocyclitis, or uveitis) is common in pauciarticular JRA. It often doesn't cause symptoms and may only be detectable through an eye exam which should be done several times per year in children with this type of JRA.

Symptoms of polyarticular JRA

Polyarticular JRA (5 or more joints affected) may have mild or no whole-body symptoms (such as weakness, tiredness, or fever).

Polyarticular JRA often begins in larger joints (such as the knee or hip) but may also start in smaller joints, such as the hands and wrists. It affects the same joints in the same way on both sides of the body (symmetric). Arthritis in the spine, neck, and jaw are more common in this type of JRA. Children who have arthritis in the jaw (temporomandibular joint) may have jaw growth abnormalities that could lead to eating problems. Small bumps under the skin (rheumatoid nodules) in pressure areas, such as the elbow or back of heel, are common in children with RF-positive polyarticular JRA. Growth problems may also be seen in the affected part of the body (such as different leg lengths if one knee joint is affected).

Symptoms of systemic JRA

Systemic JRA is characterized by whole-body symptoms and fever spikes (103°F (39.44°C) to 106°F (41.11°C)) one to two times daily.

A spotty, flat, sometimes faint red or pink rash may occur with the fever. It may occur 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. Weakness, tiredness, abdominal or chest pain, and mild loss of appetite may also occur. There may be increasing early morning stiffness, with increasing number of joints affected over time.

Symptoms of eye disease

There are often no symptoms associated with inflammatory eye disease. For this reason, it is very important for a child with JRA to have eye examinations several times per year to detect developing eye problems so that treatment can be started before permanent vision problems occur.

Other conditions with similar symptomsWhat Happens

The course of juvenile rheumatoid arthritis (JRA) is unpredictable, especially during the first few years after the child is diagnosed. Symptoms may worsen or disappear without clear reason. Eventually, the pattern of symptoms becomes more predictable. In general, children with JRA have joint pain and/or swelling and stiffness early in the course of the disease. Most children have good and bad days.

Overall, the long-term outlook for the most common type of JRA (pauciarticular JRA) is fairly good. Seventy percent to ninety percent of children with JRA recover without any serious disabilities.2 Most children with juvenile rheumatoid arthritis go on to lead normal lives, with little or no serious joint damage and few activity restrictions.

The long-term outlook for JRA varies depending on the type of juvenile rheumatoid arthritis the child has.

Children with pauciarticular JRA (few joints) have a good long-term outlook. Fifty-five percent to seventy-five percent of all children affected by JRA have this type. 3 Children with polyarticular (many joints) or systemic JRA tend to have more problems long term. Fifteen percent to twenty-five percent of children with JRA have polyarticular JRA. Less than 20% of children with JRA have systemic JRA. 3

While the overall long-term outlook for children with JRA is good, some symptoms of the disease may continue into adulthood. The type of disability may range from occasional stiffness and need for pain medication and limits on physical activity to ongoing arthritis. However, for most adults who had JRA as children, the 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).

Other types of arthritis, known as spondyloarthropathies, are different from JRA and tend to have a milder course and cause fewer long-term problems than JRA.

Additional details on course

Pauciarticular JRA (4 or fewer joints)Polyarticular JRA (5 or more joints)Systemic onset JRA (whole-body symptoms)

Complications

Complications associated with JRA can include eye disease, abnormal or slowed growth or uneven growth of a limb, and severe joint deformity or damage. Complications of systemic JRA can include heart or lung problems. See the following for more information:

Inflammatory eye diseaseGrowth abnormalities (including unequal leg lengths)Other complications (neck bone, lung, and heart problems)

What Increases Your Risk

There are no clear risk factors for juvenile rheumatoid arthritis known at this time.

When to Call a Doctor

Call for immediate care if symptoms of Reye's syndrome develop in a child who has a viral illness (such as the flu or chickenpox) and is taking aspirin. Symptoms of Reye's syndrome include:

Delirium, confusion, strange behavior (such as staring, personality changes, aggressive behavior).

Drowsiness or listlessness (loss of energy or interest in surroundings), which can result from a high fever. If listlessness does not improve after the fever is reduced with acetaminophen, call a doctor.

Persistent vomiting lasting 12 hours or longer and not clearly due to stomach flu.

Call your doctor immediately if:

There is 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 loss or blurring occurs in a child who has been diagnosed with juvenile rheumatoid arthritis.

A child taking aspirin develops signs of aspirin poisoning, including ringing in the ears, dizziness, abdominal pain, or changes in his or her mental state (such as hyperactivity or lethargy).

Call your doctor if any of the following symptoms continue for more than 2 days:

A child has unexplained daily fever spikes (103°F (39.44°C) to 106°F (41.11°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 medication use (symptoms such as heartburn, nausea, 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.

It is reasonable to try home treatment (hot or cold packs, rest, 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 there is an infection in the joint.

Who to See

For initial evaluation of joint pain and other symptoms, consult with a:

Family doctor or children's doctor (pediatrician).

Nurse practitioner (NP).

Physician assistant (PA).

For additional testing and disease management, consult with a:

Children's doctor who specializes in arthritis (pediatric rheumatologist).

Doctor who specializes in eye disease (ophthalmologist).

The disease management team will usually include:

Pediatric rheumatologist.

Nurses.

Physical and occupational therapists.

Social workers or psychologists as needed.

Exams and Tests

Many conditions can cause painful, stiff joints in children. Juvenile rheumatoid arthritis (JRA) is a relatively uncommon cause of these symptoms. Most often, joint pain in children is related to an injury or overuse. Findings from the physical exam, including the pattern and nature of joint symptoms, are an important key to the diagnosis of juvenile rheumatoid arthritis.

In most cases, routine lab tests do not give results that point to an obvious diagnosis of juvenile rheumatoid arthritis (JRA). JRA may be diagnosed only after other possible causes of symptoms have been ruled out. The following tests are mainly done to see whether another medical condition is causing joint pain or whole-body (systemic) symptoms.

Routine exams and tests include the following:

Medical history and physical exam Blood tests (complete blood count [CBC], blood chemistry panel) Erythrocyte sedimentation rate (ESR, or sed rate) Urinalysis

The following tests are done if needed:

Rheumatoid factor (RF) Antinuclear antibody (ANA) X-ray

If juvenile rheumatoid arthritis is diagnosed, a slitlamp eye exam will be done to test for possible eye problems (iritis, iridocyclitis, uveitis). This test will be repeated often during the course of the condition because the eye disease associated with JRA generally has no symptoms and can lead to permanent decrease in vision or blindness.

Other tests that may be done:

Human lymphocyte antigen or HLA-B27 (gene testing) (investigational)

Other imaging tests, joint fluid analysis, and synovial biopsy are not commonly used for diagnosing juvenile rheumatoid arthritis.

Early Detection

There is no screening for juvenile rheumatoid arthritis (JRA) at this time. Screening for eye disease (iritis, iridocyclitis, uveitis) that has no symptoms is needed when JRA is diagnosed and frequently thereafter during the first few years of the disease. Since the eye inflammation associated with JRA usually does not have symptoms and because eye problems may not be related to how severe the arthritis is, eye exams several times per year are important to detect early signs and begin treatment.

Four to seven years after the start of arthritis, less frequent exams may be needed (depending on the type of JRA). Discuss the appropriate eye exam schedule with your doctor. The following screening schedule for eye disease is recommended for children who do not currently have eye problems:4

Pauciarticular JRA (few joints)

ANA-positive

Before age 7: Every 3 to 4 months Age 7 and older: Every 6 months

ANA-negative

All ages: Every 6 months Polyarticular JRA (many joints)

ANA-positive

Before age 7: Every 3 to 4 months Age 7 and older: Every 6 months

ANA-negative

All ages: Every 6 months Systemic JRA

All ages: Every 12 months

Treatment Overview

A child with juvenile rheumatoid arthritis (JRA) will likely be treated with a combination of medications and physical therapy. The goals of treatment are to lessen the child's joint pain, improve his or her movement, and allow the child to grow and develop as normally as possible. Most children with JRA do not go on to have adult rheumatoid arthritis or other long-term problems related to JRA.

Children with pauciarticular juvenile rheumatoid arthritis can often be treated with mild, safe nonsteroidal anti-inflammatory drugs (NSAIDs); physical therapy; and occasional corticosteroid injections in the joint. For children with polyarticular JRA, NSAIDs, methotrexate, and possibly low-dose corticosteroids or corticosteroid joint injections may be used. Few children need stronger drugs (disease-modifying antirheumatic drugs, or DMARDs), systemic corticosteroids, or surgery and most do not have lifelong problems related to juvenile rheumatoid arthritis.

Methotrexate, a DMARD, has been found to be the safest, most effective second-line medication for children with JRA. Surgery is reserved for children with severe juvenile rheumatoid arthritis who have not responded to medication and physical therapy.

Exercise is an important part of treatment for a child with JRA. A child's arthritic joints must be exercised regularly and moved through their full range of motion to prevent stiffening or deformity. Gentle stretching and strengthening exercises can be beneficial for joints affected by JRA. Many children don't want to move painful joints and need encouragement to continue physical therapy.

The long-term outlook for children with JRA is good, although it varies depending on the type of JRA the child has. For instance, children with pauciarticular JRA (4 or fewer joints) have a good long-term outlook. Children with polyarticular JRA (5 or more joints) tend to have more problems long term.

In many children (50% to 70%), JRA symptoms improve or go away before adulthood. Others continue to have mild joint symptoms as adults, but with very few restrictions on activity.2 Most children with JRA will not have severely disabling or crippling arthritis. However, a few will have a worse course that leads to disability as adults.

What to Think About

Even when juvenile rheumatoid arthritis is uncomplicated, the affected child still needs many years of medical therapy. It is very important that you and your child have a good working relationship with the medical team involved in your child's care. It is also important to know what to expect from treatments and understand the importance of self-care (exercise, taking medications as prescribed).

Annual flu shots are recommended for children who are on long-term aspirin therapy. Children on long-term aspirin therapy who develop chickenpox or influenza are at risk for developing Reye's syndrome.

Prevention

The cause of juvenile rheumatoid arthritis (JRA) is currently unknown and there is no way to prevent it. The self-care methods listed below may help prevent complications and make managing the illness a bit easier.

Preventing joint pain and swellingPreventing morning stiffnessHome Treatment

Living with juvenile rheumatoid arthritis (JRA) often means making lifestyle changes and adjustments when arthritis flares up. Sometimes this can be frustrating for both you and your child. It is important to work closely with your child's doctors and other health professionals for the best results.

Exercise Balancing rest and activity Taking medications Assistive devices School issues

Family and patient education are very important for managing a long-term illness. Overall, juvenile rheumatoid arthritis (JRA) has a good long-term outlook. The outlook is even better when you and your child are active in its management. If you understand the disease, you and your child will have less fear, make better decisions, and have better results.

Medications

Most children with juvenile rheumatoid arthritis (JRA) need to take medication to reduce inflammation and pain and to help prevent increasing damage to the joints. When inflammation and pain are controlled, the child is more willing to do joint exercises to improve joint strength and prevent loss of movement.

Children with pauciarticular juvenile rheumatoid arthritis can often be treated with mild, safe nonsteroidal anti-inflammatory drugs (NSAIDs); physical therapy; and occasional corticosteroid injections in the joint. For children with polyarticular JRA, NSAIDs, methotrexate, and possibly low-dose corticosteroids or corticosteroid joint injections may be used. Few children need stronger drugs (disease-modifying antirheumatic drugs, or DMARDs), systemic corticosteroids, or surgery and most do not have lifelong problems related to juvenile rheumatoid arthritis.

Methotrexate, a DMARD, has been found to be the safest, most effective second-line medication for children with JRA. Surgery is reserved for children with severe juvenile rheumatoid arthritis who have not responded to medication and physical therapy.

Although milder NSAIDs have few side effects, they may not be enough to control a child's symptoms. If symptoms are not controlled, permanent joint damage may occur. For this reason, some doctors may begin treatment with methotrexate.

Medication Choices

Many different medications are used to treat juvenile rheumatoid arthritis (JRA). There is no single medication that works for every person. It may take some time to find the right medication or combination of medications that best controls a child's symptoms. Treatment is individualized for each child by his or her doctor and parents while considering effectiveness, side effects, cost, and severity of disease.

Nonsteroidal anti-inflammatory drugs (NSAIDs) are usually the first drugs tried to control symptoms in children with JRA. If symptoms respond well to NSAIDs and physical therapy, additional medications are not needed.5

If symptoms are not well controlled with NSAIDs, methotrexate (a stronger medication) is often used successfully.5 Methotrexate and other second-line medications are referred to as disease-modifying antirheumatic drugs, or DMARDs.

Medications used to treat JRA

Nonsteroidal anti-inflammatory drugs (NSAIDs) Disease-modifying antirheumatic drugs or DMARDs Corticosteroids

DMARDs include:

MethotrexateSulfasalazineTNF-alpha inhibitors (such as etanercept [Enbrel])

Other medications rarely used

Antimalarials (such as hydroxychloroquine sulfate [Plaquenil] or chloroquine phosphate [Aralen]) Gold saltsD-penicillamine (such as Cuprimine, Depen) Experimental therapies

Medications used to treat inflammatory eye disease

MethotrexateCorticosteroid eyedropsMydriatics

What to Think About

It is impossible to predict whether a child will improve with a certain medication. Successful drug treatment varies from child to child. Several different medications 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 medication to show effect, and symptoms may continue during that time.

Annual flu shots are recommended for children who are on long-term aspirin therapy. Children on long-term aspirin therapy who develop chicken pox or influenza are at risk for developing Reye's syndrome.

Very few cases of Reye's syndrome have been reported in children with chronic arthritis who were being treated with aspirin. Although the risk is real, Reye's syndrome is rare.

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 considered if the child has not improved with medication or physical therapy and the child is unable to walk or use his arms or hands normally.

Surgery Choices

When surgery to correct joint deformity is needed, the more commonly used procedures include the following:

Soft tissue releases of contracturesTotal joint replacement Considerations for total joint replacement in children

Other surgical procedures that have been used in children with juvenile rheumatoid arthritis (JRA) but are recommended only in selected cases include the following:

Synovectomy or tenosynovectomy (rarely used) OsteotomyEpiphysiodesisArthrodesis (joint fusion)

What to Think About

Surgery should be considered only after other options, especially physical therapy, have been fully explored without success.

The main considerations for surgery in children 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 the child with JRA have a normal lifestyle and develop normally.

Other Treatment Choices

Physical and occupational therapy

Ultrasound therapySplinting (wrist and hand, knee, ankle)Serial casting (knees, ankles, wrists, fingers, elbows)Shoe lifts or inserts

Pain management for children with arthritis

Behavioral modification for chronic painHeat, cold, or water therapyStretching and strengthening exercises

Complementary medicine therapies for pain management

MassageGuided imagery

What to Think About

Physical therapy is a vital component of the successful management of juvenile rheumatoid arthritis. It is important that the child understand why he or she needs physical therapy (if possible) and that he or she be very involved in it. A good relationship with the physical therapist is also helpful. Since most of the exercises will be done at home, parents and other caretakers need to be involved.

Other Places to Get Help Organization

National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC), National Institutes of Health

1 AMS Circle

Bethesda, MD 20892-3675

Phone:

(301) 495-4484

Fax:

(301) 718-6366

TDD:

(301) 565-2966

Web Address:

http://www.nih.gov/niams (accessed: January 22, 2001)

The clearinghouse provides educational materials about arthritis and musculoskeletal and skin diseases to the public and health professionals.

The Arthritis Foundation (AF)

1330 West Peachtree Street

Atlanta, GA 30309

Phone:

1-800-283-7800

Web Address:

http://www.arthritis.org (accessed: January 17, 2001)

This organization provides information and extensive resources about all aspects of arthritis.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health

1 AMS Circle

Bethesda, MD 20892-3675

Phone:

(301) 495-4484

Phone:

1-877-2222-NIAMS (1-877-226-4267)

Fax:

(301) 718-6366

E-mail:

niamsinfo@...

Web Address:

http://www.niams.nih.gov (accessed: February 6, 2002)

This institute provides information sources and educational materials concerning arthritis and musculoskeletal and skin diseases to the public and health professionals.

References

Citations

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Pachman LM, Poznanski AK (1997). Juvenile (rheumatoid) arthritis. In WJ Koopman, ed., Arthritis and Allied Conditions: A Textbook of Rheumatology, 13th ed., vol. 1, pp. 1156-1173. Baltimore: & Wilkins.

Mosca VS, Sherry DD (1990). Juvenile rheumatoid arthritis and the seronegative spondyloarthropathies. In RT sy, ed., Lovell and Winter's Pediatric Orthopaedics, 3rd ed., pp. 297-324. Philadelphia: J.B. Lippincott Company.

Giannini EH, Petty RE (1997). Treatment of juvenile rheumatoid arthritis. In WJ Koopman, ed., Arthritis and Allied Conditions: A Textbook of Rheumatology, 13th ed., vol. 1, pp. 1179-1193. Baltimore: & Wilkins.

Mosca VS, Sherry DD (1990). Juvenile rheumatoid arthritis and the seronegative spondyloarthropathies. In RT sy, ed., Lovell and Winter's Pediatric Orthopaedics, 3rd ed., pp. 297-324. Philadelphia: J.B. Lippincott Company.

Credits

Author

Geri Metzger

Editor

Kathleen M. Ariss, MS

Primary Medical Reviewer

J. Sexton, MD- Pediatrics

Specialist Medical Reviewer

Stanford M. Shoor, MD- Rheumatology