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Juvenile Arthritis

How Can the Family Help a Child Live Well With Juvenile Arthritis?

What Is Juvenile Arthritis?

"Arthritis" means joint inflammation. This term refers to a group of diseases that cause pain, swelling, stiffness, and loss of motion in the joints. Arthritis is also used more generally to describe the more than 100 rheumatic diseases that may affect the joints but can also cause pain, swelling, and stiffness in other supporting structures of the body such as muscles, tendons, ligaments, and bones. Some rheumatic diseases can affect other parts of the body, including various internal organs. Juvenile arthritis (JA) is a term often used to describe arthritis in children. Children can develop almost all types of arthritis that affect adults, but the most common type that affects children is juvenile idiopathic arthritis.


Both juvenile idiopathic arthritis (JIA) and juvenile rheumatoid arthritis (JRA) are classification systems for chronic arthritis in children. The juvenile rheumatoid arthritis classification system was developed decades ago and had three different subtypes: polyarticular, pauciarticular, and systemic-onset. More recently, pediatric rheumatologists throughout the world developed the juvenile idiopathic arthritis classification system, which includes more types of chronic arthritis that affect children. This classification system also provides a more accurate separation of the three juvenile rheumatoid arthritis subtypes.

Prevalence statistics for juvenile arthritis vary, but according to a 2008 report from the National Arthritis Data Workgroup, about 294,000 children age 0 to 17 are affected with arthritis or other rheumatic conditions.

What Is Juvenile Idiopathic Arthritis?

Juvenile idiopathic arthritis is currently the most widely accepted term to describe various types of chronic arthritis in children.

In general, the symptoms of juvenile idiopathic arthritis include joint pain, swelling, tenderness, warmth, and stiffness that last for more than 6 continuous weeks. It is divided into seven separate subtypes, each with characteristic symptoms:

  1. Systemic juvenile idiopathic arthritis (formerly known as systemic juvenile rheumatoid arthritis). A patient has arthritis with, or that was preceded by, a fever that has lasted for at least 2 weeks. It must be documented as an intermittent fever, spiking for at least 3 days, and it must be accompanied by at least one or more of the following:
    • Generalized enlargement of the lymph nodes.
    • Enlargement of the liver or spleen.
    • Inflammation of the lining of the heart or the lungs (pericarditis or pleuritis).
    • The characteristic rheumatoid rash, which is flat, pale, pink, and generally not itchy. The individual spots of the rash are usually the size of a quarter or smaller. They are present for a few minutes to a few hours, and then disappear without any changes in the skin. The rash may move from one part of the body to another.
  2. Oligoarticular juvenile idiopathic arthritis (formerly known as pauciarticular juvenile rheumatoid arthritis). A patient has arthritis affecting one to four joints during the first 6 months of disease. Two subcategories are recognized:
    • Persistent oligoarthritis, which means the child never has more than four joints involved throughout the disease course.
    • Extended oligoarthritis, which means that more than four joints are involved after the first 6 months of the disease.
  3. Polyarticular juvenile idiopathic arthritis-rheumatoid factor negative (formerly known as polyarticular juvenile rheumatoid arthritis-rheumatoid factor negative). A patient has arthritis in five or more joints during the first 6 months of disease, and all tests for rheumatoid factor (proteins produced by the immune system that can attack healthy tissue, which are commonly found in rheumatoid arthritis and juvenile arthritis) are negative.
  4. Polyarticular juvenile idiopathic arthritis-rheumatoid factor positive (formerly known as polyarticular rheumatoid arthritis-rheumatoid factor positive). A patient has arthritis in five or more joints during the first 6 months of the disease. Also, at least two tests for rheumatoid factor, at least 3 months apart, are positive.
  5. Psoriatic juvenile idiopathic arthritis. Patients have both arthritis and psoriasis (a skin disease), or they have arthritis and at least two of the following:
    • inflammation and swelling of an entire finger or toe (this is called dactylitis)
    • nail pitting or splitting
    • a first-degree relative with psoriasis.
  6. Enthesitis-related juvenile idiopathic arthritis. The enthesis is the point at which a ligament, tendon, or joint capsule attaches to the bone. If this point becomes inflamed, it can be tender, swollen, and painful with use. The most common locations are around the knee and at the Achilles tendon on the back of the ankle. Patients are diagnosed with this juvenile idiopathic arthritis subtype if they have both arthritis and inflammation of an enthesitis site, or if they have either arthritis or enthesitis with at least two of the following:
    • inflammation of the sacroiliac joints (at the bottom of the back) or pain and stiffness in the lumbosacral area (in the lower back)
    • a positive blood test for the human leukocyte antigen (HLA) B27 gene
    • onset of arthritis in males after age 6 years
    • a first-degree relative diagnosed with ankylosing spondylitis, enthesitis-related arthritis, or inflammation of the sacroiliac joint in association with inflammatory bowel disease or acute inflammation of the eye.
  7. Undifferentiated arthritis. A child is said to have this subtype of juvenile idiopathic arthritis if the arthritis manifestations do not fulfill the criteria for one of the other six categories or if they fulfill the criteria for more than one category.

What Causes Juvenile Arthritis?

Most forms of juvenile arthritis are autoimmune disorders, which means that the body's immune system-which normally helps to fight off bacteria or viruses-mistakenly attacks some of its own healthy cells and tissues. The result is inflammation, marked by redness, heat, pain, and swelling. Inflammation can cause joint damage. Doctors do not know why the immune system attacks healthy tissues in children who develop juvenile arthritis. Scientists suspect that it is a two-step process. First, something in a child's genetic makeup gives him or her a tendency to develop juvenile arthritis; then an environmental factor, such as a virus, triggers the development of the disease.

Not all cases of juvenile arthritis are autoimmune, however. Recent research has demonstrated that some people, such as many with systemic arthritis, have what is more accurately called an autoinflammatory condition. Although the two terms sound somewhat similar, the disease processes behind autoimmune and autoinflammatory disorders are different.

When the immune system is working properly, foreign invaders such as bacteria and viruses provoke the body to produce proteins called antibodies. Antibodies attach to these invaders so that they can be recognized and destroyed. In an autoimmune reaction, the antibodies attach to the body's own healthy tissues by mistake, signaling the body to attack them. Because they target the self, these proteins are called autoantibodies.

Like autoimmune disorders, autoinflammatory conditions also cause inflammation. And like autoimmune disorders, they also involve an overactive immune system. However, autoinflammation is not caused by autoantibodies. Instead, autoinflammation involves a more primitive part of the immune system that in healthy people causes white blood cells to destroy harmful substances. When this system goes awry, it causes inflammation for unknown reasons. In addition to inflammation, autoinflammatory diseases often cause fever and rashes.

What Are Its Symptoms and Signs?

The most common symptom of all types of juvenile arthritis is persistent joint swelling, pain, and stiffness that is typically worse in the morning or after a nap. The pain may limit movement of the affected joint, although many children, especially younger ones, will not complain of pain. Juvenile arthritis commonly affects the knees and the joints in the hands and feet. One of the earliest signs of juvenile arthritis may be limping in the morning because of an affected knee. Besides joint symptoms, children with systemic juvenile arthritis have a high fever and a skin rash. The rash and fever may appear and disappear very quickly. Systemic arthritis also may cause the lymph nodes located in the neck and other parts of the body to swell. In some cases (fewer than half), internal organs including the heart and (very rarely) the lungs, may be involved.

Eye inflammation is a potentially severe complication that commonly occurs in children with oligoarthritis but can also be seen in other types of juvenile arthritis. All children with juvenile arthritis need to have regular eye exams, including a special exam called a slit lamp exam. Eye diseases such as iritis or uveitis can be present at the beginning of arthritis but often develop some time after a child first develops juvenile arthritis. Very commonly, juvenile arthritis-associated eye inflammation does not cause any symptoms and is found only by performing eye exams.

Typically, there are periods when the symptoms of juvenile arthritis are better or disappear (remissions) and times when symptoms "flare," or get worse. Juvenile arthritis is different in each child; some may have just one or two flares and never have symptoms again, while others may experience many flares or even have symptoms that never go away.

Some children with juvenile arthritis have growth problems. Depending on the severity of the disease and the joints involved, bone growth at the affected joints may be too fast or too slow, causing one leg or arm to be longer than the other, for example, or resulting in a small or misshapen chin. Overall growth also may be slowed. Doctors are exploring the use of growth hormone to treat this problem. Juvenile arthritis may also cause joints to grow unevenly.

How Is It Diagnosed?

To be classified as juvenile arthritis, symptoms must have started before age 16. Doctors usually suspect juvenile arthritis, along with several other possible conditions, when they see children with persistent joint pain or swelling, unexplained skin rashes, and fever associated with swelling of lymph nodes or inflammation of internal organs. A diagnosis of juvenile arthritis also is considered in children with an unexplained limp or excessive clumsiness.

No single test can be used to diagnose juvenile arthritis. A doctor diagnoses juvenile arthritis by carefully examining the patient and considering his or her medical history and the results of tests that help confirm juvenile arthritis or rule out other conditions. Specific findings or problems that relate to the joints are the main factors that go into making a juvenile arthritis diagnosis.


When diagnosing juvenile arthritis, a doctor must consider not only the symptoms a child has but also the length of time these symptoms have been present. Joint swelling or other objective changes in the joint with arthritis must be present continuously for at least 6 weeks for the doctor to establish a diagnosis of juvenile arthritis. Because this factor is so important, it may be useful to keep a record of the symptoms and changes in the joints, noting when they first appeared and when they are worse or better.

Family History

It is very rare for more than one member of a family to have juvenile arthritis. But children with a family member who has juvenile arthritis are at a slightly increased risk of developing it. Research shows that juvenile arthritis is also more likely in families with a history of any autoimmune disease. One study showed that families of children with juvenile arthritis are more likely to have a member with an autoimmune disease such as rheumatoid arthritis, multiple sclerosis, or thyroid inflammation (Hashimoto's thyroiditis) than are families of children without juvenile arthritis. For that reason, having an autoimmune disease in the family may raise the doctor's suspicions that a child's joint symptoms are caused by juvenile arthritis or some other autoimmune disease.

Laboratory Tests

Laboratory tests, usually blood tests, cannot alone provide the doctor with a clear diagnosis. But these tests can be used to help rule out other conditions and classify the type of juvenile arthritis that a patient has. Blood samples may be taken to test for anti-CCP antibodies, rheumatoid factor, and antinuclear antibodies, and to determine the erythrocyte sedimentation rate (ESR), described below.

X Rays

X rays are needed if the doctor suspects injury to the bone or unusual bone development. Early in the disease, some x rays can show changes in soft tissue. In general, x rays are more useful later in the disease, when bones may be affected.

Other Tests

Because there are many causes of joint pain and swelling, the doctor must rule out other conditions before diagnosing juvenile arthritis. These include physical injury, bacterial or viral infection, Lyme disease, inflammatory bowel disease, lupus, dermatomyositis, and some forms of cancer. The doctor may use additional laboratory tests to help rule out these and other possible conditions.

Who Treats It?

Treating juvenile arthritis often requires a team approach, encompassing the child and his or her family and a number of different health professionals. Ideally, the child's care should be managed by a pediatric rheumatologist, who is a doctor who has been specially trained to treat the rheumatic diseases in children. However, many pediatricians and "adult" rheumatologists also treat children with juvenile arthritis. Because there are relatively few pediatric rheumatologists and they are mainly concentrated at major medical centers in metropolitan areas, children who live in smaller towns and rural areas may benefit from having a doctor in their town coordinate care through a pediatric rheumatologist. Many large centers now conduct outreach clinics, in which doctors and a supporting team travel from large cities to smaller towns for 1 or 2 days to treat local patients.

Other members of your child's health care team may include:

How Is It Treated?

The main goals of treatment are to preserve a high level of physical and social functioning and maintain a good quality of life. To achieve these goals, doctors recommend treatments to reduce swelling, maintain full movement in the affected joints, relieve pain, and prevent, identify, and treat complications. Most children with juvenile arthritis need a combination of medication and nonmedication treatments to reach these goals.

Following are some of the most commonly used treatments.

Medication Treatments

All medicines can have side effects. Some medicines and side effects are mentioned in this publication. Some side effects may be more severe than others. You should review the package insert that comes with your medicine and ask your health care provider or pharmacist if you have any questions about the possible side effects.

 Warning: Side effects of NSAIDs include stomach problems; skin rashes; high blood pressure; fluid retention; and liver, kidney, and heart problems. The longer a person uses NSAIDs, the more likely he or she is to have side effects, ranging from mild to serious. Many other drugs cannot be taken when a patient is being treated with NSAIDs, because NSAIDs alter the way the body uses or eliminates these other drugs. Check with your health care provider or pharmacist before you take NSAIDs. NSAIDs should only be used at the lowest dose possible for the shortest time needed.

Treatments Without Medication

How Can the Family Help a Child Live Well With Juvenile Arthritis?

Juvenile arthritis affects the entire family, all of whom must cope with the special challenges of this disease. Juvenile arthritis can strain a child's participation in social and after-school activities and make schoolwork more difficult. Family members can do several things to help the child physically and emotionally.

Do These Children Have to Limit Activities?

Although pain sometimes limits physical activity, exercise is important for reducing the symptoms of juvenile arthritis and maintaining function and range of motion of the joints. Most children with juvenile arthritis can take part fully in physical activities and selected sports when their symptoms are under control. During a disease flare, however, the doctor may advise limiting certain activities, depending on the joints involved. Once the flare is over, the child can start regular activities again.

Swimming is particularly useful because it uses many joints and muscles without putting weight on the joints. A doctor or physical therapist can recommend exercises and activities.

What Research Is Being Conducted on Juvenile Arthritis?

Researchers keep trying to improve existing treatments for children and find new medicines that will work better with fewer side effects. That effort received a major boost with the passage of the Pediatric Research Equity Act of 2003, which requires drugs that might be used in children to be tested in children. As a result of the act, increasing numbers of medications are being tested for safety and effectiveness in children. Consequently, doctors will have more information on appropriate medications and doses to prescribe for their pediatric patients.

Scientists supported by the National Institutes of Health (NIH) are investigating the possible causes of juvenile arthritis. Researchers suspect that both genetic and environmental factors are involved in development of the disease, and they are studying these factors in detail. To help explore the role of genetics, the National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) has a research registry for families in which two or more siblings have juvenile arthritis.

Eventually, gene therapy, or therapy based on the functioning of genes, may be used to treat pediatric rheumatic disorders by monitoring children's response to treatment or by predicting who is most likely to respond to a particular treatment regimen.

One recent NIAMS-supported study-the largest collaborative study of juvenile idiopathic arthritis to date-identified 14 genes linked to juvenile idiopathic arthritis and confirmed three previously discovered genes. The study also suggested that another 11 genetic regions might be involved in the disease.

Other areas of research supported by the NIH are widely varied and include studies of the following:


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