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Juvenile Rheumatoid Arthritis

(JRA; Juvenile Chronic Polyarthritis; Still’s Disease)

Definition

Juvenile rheumatoid arthritis (JRA) is a disease of the joints in children. It can effect a child over a long period of time. JRA often starts before the child is 16 years old.
In JRA, the joint to become red and swollen. It will make the joint painful and difficult to move. JRA can also lead to long term damage to the joint. For some, JRA can interfere with the child's growth and development.
There are five major types of JRA:
  • Pauciarticular JRA—four or less joints are affected in the first 6 months of illness
  • Polyarticular JRA—five or more joints are affected in the first 6 months of illness
  • Systemic onset JRA (also called Still’s disease)—affects the entire body, least common type of JRA
  • Enthesitis associated arthritis—there is also swelling of the tendon at the bone
  • Psoriatic arthritis—associated with a skin disease called psoriasis
JRA can be a serious condition. Your child will need care from a doctor. The sooner JRA is treated, the better the outcome.
Rheumatoid Arthritis
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Causes

JRA is caused by a problem of the immune system. The normal job of the immune system is to find and destroy items that should not be in the body, like viruses. With JRA, the immune system attacks the healthy tissue in the joint. It is not clear why this happens. The immune system problems may be caused by genetics and/or factors in the environment.

Risk Factors

There are no clear risk factors for JRA. Factors that may be associated with some types of JRA include:
  • Girls are more likely to get JRA than boys
  • Family history of:
  • Arthritis and a family history of psoriasis in a first-degree relative (for psoriatic arthritis)

Symptoms

JRA symptoms may be mild or severe. If your child has any of these, do not assume it is due to JRA. These symptoms may be caused by other, less serious conditions. See your doctor if your child has any of these symptoms:
  • Joint stiffness, especially in the morning or after periods of rest
  • Pain, swelling, tenderness, or weakness in the joints
  • Fever
  • Weight loss
  • Fatigue or irritability
  • Swelling in the eye—especially associated with eye pain, redness, or sensitivity to light
  • Swollen lymph nodes
  • Growth problems, such as:
    • Growth that is too fast or too slow in one joint (may cause one leg or arm to be longer than the other)
    • Joints grow unevenly, off to one side
    • Overall growth may be slowed
Some symptoms are specific to each type of JRA. For example:
  • Symptoms common with pauciarticular JRA include:
    • Problems most often found in large joints. These joints include knees, ankles, wrists, and elbows.
    • If left-side joint is affected the right-side similar joint will not be affected. For example, if right knee is affected the left knee will be healthy.
    • May also have swelling and pain at on the tendons and ligaments attached to the bone.
  • Symptoms common with polyarticular JRA include:
    • Problems found most often in small joints of the fingers and hands. May also affect weight-bearing joints like the knees, hips, ankles, and feet.
    • Joints on both sides of the body are affected. For example if left hand is affected the right hand will also be affected
    • May also have a blood disorder called anemia . This is an abnormally low number of red blood cells.
    • One type of polyarticular JRA may occur with:
      • A low-grade fever
      • Nodules—bumps on parts of body that receive a lot of pressure such as elbows
  • Symptoms common with systemic onset JRA include:
    • Some of the first signs may be a high fever, chills, and a rash on the thighs and chest. May appear on and off for weeks or months.
    • May have swelling in heart, lungs, and surrounding tissues.
    • Lymph nodes, liver, and/or spleen may become enlarged.
  • Children with enthesitis arthritis often have tenderness over joint where the pelvis and spine.
  • Children with psoriatic arthritis often have finger or toe swelling. There may also be damage on fingernails.
Often, there are remissions and flare-ups. Remission is a time when the symptoms are better or disappear. Flare-ups are times when symptoms become worse.

Diagnosis

More than one test may be needed to diagnose JRA. The tests will also help to eliminate the possibility of other diseases.
Your doctor will ask about your child’s symptoms. You will also be asked about your family medical history. A physical exam will be done. Your child may be referred to a special doctor if JRA is suspected. The specialist is a doctor that focuses on diseases of the joints.
Tests that may be ordered include:
  • Images of joints and bones such as x-rays
  • Laboratory tests on blood, urine, and/or joint fluid:
    • May be used to eliminate other health conditions such as physical injury, infections, other autoimmune diseases, and some forms of cancer
    • Can also help to determine specific type of JRA
  • Eye examinations—to look for swelling in the eye

Treatment

Talk with your doctor about the best plan for your child. The plan will work to control swelling, relieve pain, and control joint damage. The goal is to keep a high level of physical and social function. This will help keep a good quality of life. Treatment options include the following:

Medication

There are several types of medication that may be used:
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)—to help swelling and pain
  • Disease-modifying antirheumatic drugs (DMARDs)—to slow the progression of the disease
  • Tumor necrosis factor (TNF) blockers —to decrease swelling, pain, and joint stiffness
  • Interleukin inhibitors—to reduces disease activity
  • Corticosteroids through IV or by mouth—for swelling
  • Steroid injections into the joint—may help relieve swelling and pain in some children

Physical Therapy

Exercise is done to keep the muscles around the joint strong. Strong nearby muscles will support the joint. It also help to recover the range of motion of the joints. Normal daily activity are encouraged. Non-contact sports and recreational activities may be good options. Physical activities can also help boost a child's confidence in their physical abilities.
Physical therapy may be needed. This will help to make the muscles strong and keep the joints moving well.

Maintenance Devices

Splints and other devices may be recommended. They may be worn to keep bone and joint growth normal. Some joints may get stuck in a bent position. These devices can help prevent this.

Prevention

There is no known way to prevent JRA.

RESOURCES

American College of Rheumatology http://www.rheumatology.org/

Arthritis Foundation http://www.arthritis.org/

CANADIAN RESOURCES

The Arthritis Society http://www.arthritis.ca/

Health Canada http://www.hc-sc.gc.ca/index%5Fe.html/

References

Behrman RE, Kliegman R, Jenson H. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, PA: Saunders; 2007.

Firestein G, Kelley W. Kelley’s Textbook of Rheumatology. 8th ed. Philadelphia, PA: Saunders; 2008.

Hofer MF, Mouy R, Prieur AM. Juvenile idiopathic arthritides evaluated prospectively in a single center according to the Durban criteria. J Rheumatol. 2001. 28:1083.

Juvenile idiopathic arthritis (JIA) enthesitis related. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 4, 2012. Accessed August 2, 2012.

Juvenile idiopathic arthritis (JIA) oligoarticular. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 4, 2012. Accessed August 2, 2012.

Juvenile idiopathic arthritis (JIA) polyarticular. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 4, 2012. Accessed August 2, 2012.

Juvenile idiopathic arthritis (JIA) systemic-onset. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed/what.php . Updated June 4, 2012. Accessed August 2, 2012.

JAMA Patient Page. Juvenile idiopathic arthritis. JAMA . 2005;294:1722.

Petty RE, Southwood TR, Baum J, et al. Revision of the proposed classification criteria for juvenile idiopathic arthritis: Durban, 1997. J Rheumatol .1998; 25:1991.

2/5/2013 DynaMed's Systematic Literature Surveillance http://www.ebscohost.com/dynamed/what.php : De Benedetti F, Brunner HI, Ruperto N, et al. Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Eng J Med. 2012;367(25):2385-95.

Revision Information

  • Reviewer: Michael Woods
  • Review Date: 09/2012 -
  • Update Date: 02/05/2013 -

This content is reviewed regularly and is updated when new and relevant evidence is made available. This information is neither intended nor implied to be a substitute for professional medical advice. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with questions regarding a medical condition.

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