Causes

The cause of JRA is not known. It is thought to be an autoimmune illness. This means the body's immune system mistakenly attacks and destroys healthy body tissue.

JRA usually occurs before age 16. Symptoms may start as early as 6 months old.

It is divided into several types:

• Systemic (bodywide) JRA involves joint swelling or pain, fevers, and rash. It is the least common type.
• Polyarticular JRA involves many joints. This form of JRA may turn into rheumatoid arthritis. It may involve five or more large and small joints of the legs and arms, as well as the jaw and neck.
• Pauciarticular JRA involves four or less joints, most often the wrists, or knees. It also affects the eyes.

Symptoms

Symptoms of JRA may begin with a swollen joint, limping, a spiking fever, or a new rash.

Symptoms can include:

• Joint stiffness and pain
• Limited range of motion
• Warm, swollen, or red joints
• A child may stop using an affected limb or may limp

Body-wide JRA symptoms:

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

JRA can also cause eye problems called uveitis, iridocyclitis, or iritis. There may be no symptoms, or the person may have:

• Red eyes
• Eye pain, which may get worse when looking at light (photophobia)
• Vision changes

Exams 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:

• Swollen liver
• Swollen spleen
• Swollen lymph nodes

Blood tests that may be done include:

• Rheumatoid factor
• Erythrocyte sedimentation rate (ESR)
• ANA
• Complete blood count (CBC)
• HLA antigens for HLA B27

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

The health care provider may place a small needle into a swollen joint to remove fluid. This can help to find the cause of the arthritis and help relieve pain, too. Sometimes, the health care provider will inject steroids into the joint to help reduce swelling.

Other tests that may be done include:

• X-ray of a joint
• Bone scan
• 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 in the joints or body. DMARDs include:

• Methotrexate
• Biologic drugs, such as such as etanercept, infliximab, and related drugs

It is important for children with JRA to stay active and keep their muscles strong.

Exercise will help keep their muscles and joints strong and mobile.

• Walking, bicycling, and swimming may be good activities.
• Children should learn to warm up before exercising.
• Talk to the doctor or physical therapist about exercises to do when your child is having pain.

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

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

Outlook (Prognosis)

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

The greater the number of joints affected, the more severe the disease and the less likely that the symptoms will eventually go into total remission.

Children with JRA who have many joints involved, or who have a positive rheumatoid factor are more likely to have chronic pain, disability, and poor school attendance. The greater the number of joints affected, the more severe the disease and the less likely that the symptoms will eventually go into total remission.

For additional information and resources, see: Arthritis support group

Possible 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

When to Contact a Medical Professional

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.

Alternative Names

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

References

Long AR, Rouster-Stevens KA. The role of exercise therapy in the management of juvenile idiopathic arthritis. Curr Opin Rheumatol. 2010 Mar;22(2):213-7.

Prince FH, Otten MH, van Suijlekom-Smit LW. Diagnosis and management of juvenile idiopathic arthritis. BMJ. 2010 Dec 3;341:c6434.

Ruperto N, Lovell DJ, Quartier P, et al; Paediatric Rheumatology International Trials Organization and the Pediatric Rheumatology Collaborative Study Group. Long-term safety and efficacy of abatacept in children with juvenile idiopathic arthritis. Arthritis Rheum. 2010 Jun;62(6):1792-802.

Update Date: 4/28/2012

Reviewed by: Ariel D. Teitel, M.D., M.B.A., Chief, Division of Rheumatology, St. Vincent’s Hospital, New York, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.