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Arthritis: General Concepts > Juvenile Rheumatoid Arthritis

What Is Juvenile Rheumatoid Arthritis?

Arthritis is a chronic condition that involves inflammation of the joints and the connective tissue around the joints. Juvenile rheumatoid arthritis (JRA) is a kind of arthritis that occurs in children before age 16. It is sometimes called juvenile chronic polyarthritis or Still’s disease. Mostly, it involves an inflammation of the connective tissue.

With rheumatoid arthritis, the body's immune system to produces a chemical that attacks and destroys the protective cartilage covering the joint surface. The normally smooth and shiny joint lining, known as synovium, becomes inflamed and swollen. Over time, this lining thickens and the affected joints begin to disintegrate. As the disease progresses, the bones of the joints may move out of their correct positions.

The arthritis associated with JRA can come on quickly, or it can develop slowly over a period of time. The disease can affect a large number of joints in the body, or it may be confined to only one or two joints, such as to the ankle and knee. The number of joints involved is one way doctors classify the kind of JRA a child has.

While JRA is seldom life threatening, it is a complicated disease, and its effects are difficult to predict. The most common problem for patients and their families is learning how to cope with long bouts of a chronic condition that can be very painful and cause deformities. There is often marked improvement, however, or even total remission of the disease when the child reaches puberty. And three out of every four patients with the disease eventually enter remission with very little damage that lasts. JRA can lead to serious complications, including a decrease or loss of vision and total joint destruction of the major weight-bearing joints.

In addition to its effect on the body’s joints, JRA can affect a number of other systems in the body. It can, for instance, affect the heart and the lungs and the tissue around those organs. It can also have an effect on the eyes or on the skin, and it can cause fevers, a rash, and an enlarged spleen. When the disease is in its active state, it may affect the child’s growth.

Causes of Juvenile Rheumatoid Arthritis?


JRA is an autoimmune disorder, in which the body mistakenly identifies some of its own cells and tissues as foreign. The immune system, which normally fights off bacteria or viruses, begins to attack healthy cells and tissue instead.

Researchers suspect that the development of JRA is a two-step process. First, something in a child's genetic makeup gives them a tendency to develop the disease. Then an environmental factor, such as a virus or traumatic injury, triggers the onset of illness.

Common risk factors include:
  • a family history of JRA, appearing in several generations or in children who are siblings
  • an infection such as the flu, Lyme disease, infectious mononucleosis, or a recent immunization for rubella or DPT (diptheria-pertussis- tetanus)
  • stress, such as a divorce, separation, death in the family, or adoption
  • a severe injury or physical trauma
JRA does seem to affect girls four times more often than boys, and it is most likely to occur at two different stages in a child’s life — between the ages of 2 and 5 and the ages of 9 and 12.

Symptoms of Juvenile Rheumatoid Arthritis?

Like adult onset rheumatoid arthritis, JRA involves inflammation of the connective tissue. Although many young children with JRA do not complain as much of joint pain, the disease often causes redness, swelling, warmth, soreness, and the loss of motion in the joints. The most common symptom is the persistent swelling of particular joints accompanied by pain and stiffness that is worse in the morning or after a nap.

JRA may flare up quickly or develop slowly over time. Sometimes a large number of joints are involved, or as few as only one or two, such as the ankle and knee joints. One of the earliest signs of JRA may be limping in the morning because of an affected knee.

The main difference between juvenile and adult rheumatoid arthritis is that the majority of children with JRA outgrow the illness, while adults are likely to have lifelong symptoms. Another difference is that about 70 to 80 percent of all adults with rheumatoid arthritis test positive for rheumatoid factor (RF) in their blood, whereas less than half of all children with rheumatoid arthritis are RF positive. Testing positive for RF indicates an increased chance that JRA will continue into adulthood.

JRA may also affect a number of other systems in the body besides the joints. It can affect the heart and (more rarely) the lungs, as well as the tissue around those organs. Sometimes JRA results in damage to the eyes. It may cause fevers, rash, or an enlarged spleen. Because JRA develops before the skeleton is mature, JRA may affect bone development as well as the child's growth.

While JRA is seldom life threatening, it is a complex disease requiring careful management. It is almost always unpredictable and disruptive. The challenge for patients and their families is learning how to cope with the pain and difficulty of long bouts of a chronic condition during the childhood years.

General symptoms:
  • joint stiffness upon waking in the morning
  • limited range of motion
  • slow rate of growth
  • hot, swollen, painful joints
  • fever, either low grade or high spiking with chills, depending on the form of the disease
  • rheumatoid rash
  • rheumatoid nodules (lumps on the skin at sites of pressure)
Symptoms that indicate eye involvement:
  • red eyes
  • eye pain
  • photophobia
  • visual changes
Other symptoms:
  • chest pain
  • shortness of breath
  • abdominal pain
Different Types of Juvenile Rheumatoid Arthritis?

Doctors classify JRA according to the number of joints involved and the presence or absence of certain antibodies in the blood. These antibodies are special proteins made by the immune system. Three categories distinguish among forms of JRA, each of which has unique features and progresses somewhat differently:
  • Pauciarticular (paw-see-are-tick-you-lar) JRA involves four or fewer joints. The most common form of JRA, affecting about half of all children with the disease, pauciarticular JRA is most common among girls under the age of 8. It typically affects large joints, such as the knees.

  • About 20 to 30 percent of children with pauciarticular JRA develop problems with their eyesight. Up to 80 percent of those with eye disease also test positive for antinuclear antibodies or ANAs. Both ANA and JRA tend to develop at a very early age in these children.

  • Children with blood tests indicating ANA should be examined regularly by an eye doctor (an ophthalmologist) in order to treat or prevent serious problems such as iritis, the inflammation of the iris, or uveitis, inflammation of the inner eye. Although most children with pauciarticular JRA outgrow arthritis during adolescence, some continue to have problems with their eyes or recurring symptoms of joint pain.
  • Polyarticular JRA affects about 30 percent of all children with the disease. In polyarticular JRA, five or more joints are affected. The small joints of the hands and feet are most commonly involved, usually in a symmetrical fashion. Some children have enlarged lymph nodes (under the arms, for example) or develop lumps under the skin known as subcutaneous nodules. These painless lumps are usually located around the elbows, wrists, forearms, ankles, or feet, attached to the coverings of bones or other tissue.

  • Some children with polyarticular disease have a special kind of antibody in their blood called IgM rheumatoid factor, also known as RF. These children usually have a more severe form of the disease, which doctors consider to be the equivalent of adult rheumatoid arthritis.
  • Systemic JRA is the most alarming and aggressive form of the disease, affecting 20 percent of all children with JRA. This is the most likely form of JRA to affect boys. Besides causing the joints to swell, systemic JRA is characterized by intermittent or spiking fever. Another indication is a salmon-colored smooth rash with clearly defined irregular borders. This rash, seen most frequently on the face and trunk, often moves around and may appear and disappear within minutes.

  • Many children who develop systemic JRA are hospitalized during the early stages of the disease. During hospitalization many tests are run in order to arrive at a diagnosis. Blood tests on almost all children with systemic JRA are negative for both RF and ANA.

  • Sometimes known as Still's Disease, systemic JRA may also affect internal organs such as the heart, liver, spleen, and lymph nodes. Sometimes a temporary condition known as pericarditis develops, in which there is fluid inside the sac around the heart.

  • A small percentage of children with systemic JRA develop arthritis in many joints and have severe arthritis that continues into adulthood.
Treatment of Juvenile Rheumatoid Arthritis?

There are two main goals for treatment:
1. to preserve mobility and joint function
2. to provide support through a long chronic illness

A doctor diagnoses JRA by carefully examining the child, considering his or her medical history, and reading the results of laboratory tests that help rule out other conditions. X rays are needed if the doctor suspects injury to the bone or unusual bone development. Symptoms must be present for at least 6 weeks for the doctor to consider a diagnosis of JRA.

JRA is best managed using a team approach. A pediatrician, family physician, or other primary care doctor usually manages treatment, with the help of other doctors. These may include pediatric rheumatologists (doctors specializing in childhood arthritis), ophthalmologists (eye doctors), orthopaedic surgeons (bone specialists), and physiatrists (rehabilitation specialists), as well as physical and occupational therapists. Sometimes counseling is used to help the child and family cope with the emotional aspects of the illness.

The goals of treatment are to preserve a high level of physical and social functioning, allowing the child to maintain a good quality of life despite the illness. Treatment aims to reduce pain and swelling, maintain full movement in the affected joints, and identify, treat, and prevent complications. Medication and physical therapy are used to achieve these goals.
  • Nonsteroidal Anti-Inflammatory Drugs (NSAIDs) such as aspirin, ibuprofen (Motrin, Advil, Nuprin) and naproxen or naproxen sodium (Naprosyn, Aleve) are often are the first type of medication used.

  • Most doctors hesitate to treat children with aspirin because of the possibility that it will cause bleeding problems, upset stomach, liver problems, or Reye's syndrome. But for some children aspirin in the correct dose, as measured by blood test, can effectively control JRA with few serious side effects.
  • Disease-Modifying Anti-Rheumatic Drugs (DMARDs) are often used if NSAIDs do not adequately relieve symptoms. Because DMARDs may take weeks or months to slow the progress of JRA, they are often taken in combination with a NSAID.

  • Various types of DMARDs are available, but today methotrexate is most commonly used. Because only small doses of methotrexate are needed to relieve arthritis symptoms, potentially dangerous side effects rarely occur. The most serious complication is liver damage, but it can be avoided with regular blood tests and careful monitoring.
  • Corticosteroids, given either orally or intravenously, may be needed to treat serious symptoms such as inflammation of the sac around the heart (pericarditis).


Because corticosteroids can interfere with a child's normal growth and cause other side effects, such as a round face, weakened bones, and increased susceptibility to infections, their use must be carefully monitored. It is important to follow the doctor’s instructions about how to take or reduce the dosage of corticosteroids, as it can be dangerous to stop taking these medications suddenly. Physical therapy is another vital part of a child's treatment plan. Besides helping to maintain muscle tone, exercise can preserve and recover the range of motion of the joints. It also helps a child maintain self-confidence and emotional strength to cope with the ups and downs of JRA.

A physical therapist helps design an appropriate exercise program for a person with JRA, and may recommend using splints and other devices to help the joints grow evenly. Swimming is highly recommended because it uses many joints and muscles without putting weight on the body. Whirlpool treatment and the use of heat or ice is also beneficial in reducing discomfort.

Surgery may occasionally be recommended for children with JRA, when there are obvious deformities of the joints. An orthopedic surgeon can advise the patient and his or her family about the kinds of surgery that may be appropriate in helping to control the disease.

Living with Juvenile Rheumatoid Arthritis?
When a child has JRA, the entire family must learn to deal with special challenges. A child with JRA may find it difficult to participate in social and after-school activities, as well as the requirements of schoolwork. It helps to work closely with the school to develop suitable lesson plans for the child, as well as to educate the child’s teacher and classmates about JRA. Children with JRA may need to be educated at home periodically, and they may benefit from tutoring or simple adjustments in the school routine.

It is helpful if family members and teachers treat the child as normally as possible, making adjustments as needed during the course of the disease. When symptoms are under control, a child can be more active, even participating in sports. At other times, activities need to be limited.

It is important to ensure that the child receives appropriate medical care, tailored to the specific needs of the child. What works for one child may not work for another. If the medications that the doctor prescribes do not relieve symptoms or if they cause unpleasant side effects, patients and parents should discuss other choices with their medical team.

The American Juvenile Arthritis Organization runs support groups for the families of people with JRA. Support groups provide the chance to network with other young people and parents of children with JRA. The support offered by other families is often invaluable in helping a family deal with the challenges of living with juvenile rheumatoid arthritis.

The information provided herein is not intended to be a substitute for professional medical advice. You should not use this information to diagnose or treat a health problem or disease without consulting a licensed physician.

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