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Juvenile rheumatoid arthritis

Definition

Juvenile rheumatoid arthritis (JRA) is a term used to describe a common type of arthritis in children. It is a long-term (chronic) disease resulting in joint pain and swelling.

Alternative Names

Juvenile chronic polyarthritis; JRA; Still's disease; Juvenile idiopathic arthritis

Causes, incidence, and risk factors

The cause of JRA is not known. It is thought to be an autoimmune illness. In this type of condition, the body's immune system mistakenly attacks and destroys healthy body tissue.

JRA usually occurs before age 16.

It is divided into several categories:

• Systemic JRA involves joint swelling or pain, fevers, and rash. It is the least common category.
• Polyarticular JRA involves many joints. This form of JRA may turn into rheumatoid arthritis. It may involve large and small joints of the legs and arms, as well as the TMJ and cervical spine.
• Pauciarticular JRA involves only a few joints, usually the hips, knees, or ankles.

Many other medical problems can cause symptoms similar to those of juvenile rheumatoid arthritis, including:

• Lyme disease, rheumatic fever, bone infections (osteomyelitis), and other infections
• Sarcoidosis, psoriatic arthritis, vasculitis
• Inflammatory bowel disease

Symptoms

Arthritis symptoms:

• Joint stiffness when you wake up in the morning
• Joint pain
• Limited range of motion
• Joints may be warm or swollen and sometimes red
• A child may stop using an affected limb or may limp
• Back pain

Body-wide JRA symptoms:

• Fever, usually high fevers every day
• Rash (trunk and extremities) that comes and goes with the fever
• Swollen lymph nodes (glands)

JRA can also cause eye inflammation called uveitis. This problem can occur without any eye symptoms, or someone may have:

• Red eyes
• Eye pain
• Increased pain when looking at light (photophobia)
• Vision changes

Signs and tests

The physical examination may show swollen, warm, and tender joints that hurt to move. The child may have a rash. Other signs include:

• Enlarged liver
• Enlarged spleen
• Swollen lymph nodes

Blood tests may include:

• Rheumatoid factor (may be raised, but not in all patients)
• Erythrocyte sedimentation rate (ESR)
• ANA (may be high)
• Complete blood count (CBC)
• HLA antigens for HLA B27

Any or all of these blood tests may be normal in patients with JRA.

The doctor may need to tap a joint. This means putting a small needle into a joint that is swollen. This can help to find the cause of the arthritis. By removing fluid, the joint may feel better, too. Sometimes, the doctor will inject steroids into the joint to help decrease the swelling.

Other tests:

• X-ray of a joint
• X-ray of the chest
• ECG
• Eye exam by an ophthalmologist (should be done on a regular basis, even if there are no eye symptoms)

Treatment

When only a small number of joints are involved, nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen may be enough to control symptoms.

Corticosteroids may be used for more severe flare-ups to help control symptoms.

Children who have arthritis in many joints, or who have fever, rash, and swollen glands may need other medicines. These medicines are called disease-modifying antirheumatic drugs (DMARDs). They can decrease or prevent swelling or inflammation in the body. DMARDs include:

• Methotrexate is often the first drug used.
• Biologic drugs, such as such as etanercept, infliximab, and related drugs block high levels of proteins that cause inflammation.

It is important for children with JRA to stay active and keep their muscles strong. Walking, bicycling, and swimming may be good activities. Children should learn to warm up before exercising.

Support and help for children who experience sadness or anger about their arthritis is also very important.

Some children with JRA may need surgery, including joint replacement.

Support Groups

Expectations (prognosis)

JRA is seldom life threatening.

Children who have many joints involved, or who have a positive rheumatoid factor are more likely to have chronic pain and poor school attendance, and to be disabled.

Long periods with no symptoms are more common in those who have only a small number of joints involved. Many patients with JRA eventually go into remission with very little loss of function and deformity.

For additional information and resources, see arthritis support group.

Complications

• Wearing away or destruction of joints (can occur in patients with more severe JRA)
• Slow rate of growth
• Uneven growth of an arm or leg
• Loss of vision or decreased vision from chronic uveitis (this problem may be severe, even when the arthritis is not very severe)
• Anemia
• Swelling around the heart (pericarditis)
• Chronic pain, poor school attendance

Calling your health care provider

Call for an appointment with your health care provider if:

• You notice symptoms of juvenile rheumatoid arthritis
• Symptoms get worse or do not improve with treatment
• New symptoms develop

Prevention

There is no known prevention for JRA.

References

Miller ML, Cassidy JT. Juvenile rheumatoid arthritis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders Elsevier; 2007: chap 154.

Lovell DJ, Ruperto N, Goodman S, Reiff A, Jung L, Jarosova K, et al. Pediatric Rheumatology Collaborative Study Group: Pediatric Rheumatology International Trials Organisation. Adalimumab with or without methotrexate in juvenile rheumatoid arthritis. N Engl J Med. 2008;359:810-820.

Cespedes-Cruz A, Gutiérrez-Suárez R, Pistorio A, Ravelli A, Loy A, Murray KJ, et al. Pediatric Rheumatology International Trials Organization (PRINTO). Methotrexate improves the health-related quality of life of children with juvenile rheumatoid arthritis. Ann Rheum Dis. 2008;67:309-314.

Ravelli A, Martini A. Juvenile idiopathic arthritis. Lancet. 2007;369:767-778.