Juvenile rheumatoid arthritis

Alternative names 
Juvenile chronic polyarthritis; JRA; Still’s disease

Definition
Juvenile Rheumatoid Arthritis is a chronic, inflammatory disease resulting in joint pain and inflammation. It may result in joint damage. It usually occurs before age 16.

Causes, incidence, and risk factors

Juvenile Rheumatoid Arthritis (JRA) is a chronic inflammatory arthritis in children. It occurs in between 50-100 per 100,000 children in the United States. JRA is a general term for the most common types of arthritis in children. It is divided in to several categories:

  1. Systemic JRA: This form, which occurs in about 10% of cases, involves joint pain and swelling as well as fevers and rash. It is similar to Adult Still’s Disease. The cause of this form of JRA is unknown.
  2. Polyarticular JRA: This form occurs in about 40% of cases and involves multiple joints that are painful and swollen. The cause of this form of JRA is unknown. Some children may have a positive Rheumatoid Factor and evolve into Rheumatoid Arthritis.
  3. Pauciarticular JRA: This form occurs in about 50% of cases and involves only a few joints. Some of these children, in particular boys, will be HLA-B27 positive. Families with HLA-B27 are at increased risk for this arthritis.

Symptoms
Arthritis symptoms:

     
  • joint stiffness on arising in the morning  
  • limited range of motion  
  • slow rate of growth or uneven arm or leg growth  
  • hot, swollen, painful joints  
  • a child my stop using an affected limb  
  • back pain

Systemic JRA symptoms:

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

JRA can also cause eye inflammation. These symptoms include:

     
  • red eyes  
  • eye pain  
  • photophobia (increased pain when looking at a light)  
  • visual changes

Signs and tests

The physical examination shows swollen, warm, and tender joints that hurt to move. The child may have a rash. Other signs include an enlarged liver, enlarged spleen, or swollen lymph nodes.

Blood tests may include:

     
  • CBC  
  • ESR (sedimentation rate)  
  • ANA  
  • RA factor  
  • HLA antigens for HLA B27

The doctor may need to tap a joint. This means that they will put 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 ophthomologist

Treatment
The goal is to preserve mobility and joint function and support the patient and family through a long chronic illness.

Therapeutic medications include:

     
  • nonsteroidal antiinflammatory agents (NSAIDS)  
  • corticosteroids  
  • topical ophthalmic corticosteroids  
  • hydroxychloroquine  
  • immune suppressing agents, including methotrexate and new biologic medications like infliximab and etanercept.

Note: Talk to your health care provider before giving aspirin or NSAIDS to children!

Physical therapy and exercise programs may be recommended. Surgical procedures may be indicated, including joint replacement.

Expectations (prognosis)
JRA is seldom life-threatening. Long periods of spontaneous remission are typical. Often, JRA improves or goes into remission at puberty. Approximately 75% of JRA patients eventually enter remission with minimal functional loss and deformity.

The stress of illness can often be helped by joining a support group where members share common experiences and problems. See arthritis support group.

Complications

     
  • total joint destruction of the major weight-bearing joints  
  • loss of vision or decreased vision  
  • chronic spondyloarthropathy (back stiffness)

Calling your health care provider
Call for an appointment with your health care provider if you notice symptoms of juvenile rheumatoid arthritis. Also call your health care provider if symptoms worsen, do not improve with treatment, or new symptoms develop.

Prevention
There is no known prevention for JRA.

Johns Hopkins patient information

Last revised: December 3, 2012
by Levon Ter-Markosyan, D.M.D.

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