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Rheumatoid Arthritis, Juvenile

What is it?

Juvenile rheumatoid arthritis is an uncommon childhood condition characterized by persistent or recurrent joint inflammation. Joints contain a number of structures and fluids that allow for ease of movement. The ends of the bones in a joint are protected from rubbing together by an elastic cushioning material (cartilage). The entire joint is surrounded by a capsule (synovial sac). A thin layer of tissue (synovial membrane) lines the sac and secretes synovial fluid, which provides lubrication to ease movement. But inflammation may thicken, erode, or otherwise damage these structures, causing pain and limiting movement. Current thinking is that juvenile rheumatoid arthritis is an autoimmune disorder, an attack by the immune system on some of the body's own cells. Most cases of juvenile rheumatoid arthritis have clinical features significantly different from those of the type of rheumatoid arthritis seen in adults (see Rheumatoid Arthritis for specific information about that disorder). There are three main types of juvenile arthritis: pauciarticular and polyarticular juvenile rheumatoid arthritis, and Still's disease (also known as systemic juvenile arthritis). Pauciarticular juvenile rheumatoid arthritis, which accounts for approximately 40 percent of cases, causes pain, swelling, and stiffness in four or fewer joints. Polyarticular juvenile rheumatoid arthritis, a more severe form of the disease that accounts for another 40 percent of cases, causes pain, swelling, and stiffness in a number of joints throughout the skeleton. In Still's disease, which accounts for the remaining 20 percent of cases, the child may exhibit symptoms of general illness, including fever, rash, and abdominal pain that last for several weeks and precede symptoms of joint pain and stiffness by several months. Still's disease may cause inflammation of the eyes and lining of the lungs (pleura) or heart (pericardium). Unlike other forms of juvenile arthritis, Still's disease may also occasionally affect adults. Joint symptoms of juvenile rheumatoid arthritis tend to occur in episodes of several weeks, separated by remission periods when symptoms subside.With frequent or severe episodes, bone growth may be uneven or abnormal. Permanent joint deformity and functional limitation occurs in over 30 percent of affected children after 10 years of follow-up. Early-onset pauciarticular patients are typically very young girls (one to five years of age). They have the greatest risk for developing chronic eye inflammation, and have the best overall joint outcome. Late-onset pauciarticular patients (ages nine to 12) are more often boys; they often have tendinitis as well as arthritis affecting large joints (shoulders, hips, and knees) or the spine. Polyarticular juvenile rheumatoid arthritis may develop at any age, and girls are affected three times more often than boys. Still's disease may develop at any age, but the peak age of onset is one to six years of age. Boys and girls are equally affected. In many cases of Still's disease, symptoms disappear around the age of puberty. Patients with pauciarticular or polyarticular disease (approximately 40 percent) may have persistent joint symptoms. Treatment is aimed at relieving symptoms, preventing deformity, and maintaining optimal joint function throughout the duration of the disease.

What Causes It?

  • The cause of juvenile rheumatoid arthritis is unknown, but genetic factors play a role.

Prevention

  • There is no known way to prevent juvenile rheumatoid arthritis.

Diagnosis

  • Patient history and physical examination are needed. There is no specific diagnostic test for juvenile rheumatoid arthritis, and long-term observation may be necessary for definitive diagnosis.
  • Blood tests will be taken.
  • X-rays of the affected joints may be ordered.
  • Synovial fluid analysis may be performed. In this procedure a local anesthetic is administered to desensitize the affected joint, and synovial fluid is then withdrawn from the joint capsule with a needle.

How to Treat It

  • To reduce fever and treat minor pain, your doctor may prescribe large doses of aspirin or one of the many other nonsteroidal anti-inflammatory drugs (NSAIDs), such as tolmetin or naproxen.
  • Hot or cold compresses applied to the joints may offer some pain relief.
  • Creams or lotions containing capsaicin may be applied to relieve minor joint pain. Preparations containing camphor, menthol, or turpentine oil may mask pain and provide some relief from minor symptoms.
  • In severe cases, stronger medications may be prescribed to relieve pain and inflammation. The immunosuppressive drug methotrexate has been well tolerated and is effective, and is now the preferred second-line agent among most practitioners. Uncontrolled studies of sulfasalazine in children have been encouraging, but the drug has yet to be studied under double-blind, controlled conditions. Although injectable gold salt preparations have been useful, their role in treating patients has declined dramatically over the past 10 years (as the use of methotrexate has increased). Agents such as the antimalarial hydroxychloroquine, penicillamine, and oral gold salts are of little benefit.
  • Corticosteroids, such as prednisone, offer quick relief from symptoms of juvenile rheumatoid arthritis but have serious side effects; they are used only in the smallest possible doses and only when other treatments prove ineffective.Oral prednisone has an important place, however, in the treatment of many patients with Still's disease, especially those with inflammation of the lining around the heart (pericarditis) and persistent fever. Intra-articular corticosteroids may be helpful for patients with limited joint involvement.
  • Plenty of rest is required. Children with juvenile rheumatoid arthritis often need to sleep longer than average at night and may need to take naps during the day.
  • Your child's doctor may prescribe an exercise program or may advise that your child see a physical therapist.While exercise that is too vigorous may worsen symptoms, some regular activity is necessary to prevent limitation of joint movements and to prevent muscle weakness and atrophy. Gentle exercises are taught to increase the range of motion in affected joints. Some movements are easier to perform in a pool or hot tub, since water helps support the body.
  • Splints may be prescribed to relieve pain by immobilizing affected joints during severe episodes.
  • Contact a local chapter of the Arthritis Foundation for information about support groups in your area.

When to Call a Doctor

  • Make an appointment with a doctor if your child develops painful or stiff joints.