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Juvenile Rheumatoid Arthritis
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Juvenile Rheumatoid Arthritis

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

Pronounced: jew-va-nigh-el roo-MAH-toyd arth-RI-tis

En Español (Spanish Version)

Definition

Juvenile rheumatoid arthritis (JRA) is a chronic (long-lasting) disease of the joints, which starts before the age of 16 years old. Cases may range from mild to severe. The joints become inflamed, which leads to pain and/or stiffness. The inflammation can damage cartilage and bone, and in some cases cause changes in growth and development.

There are three major types of JRA:

  • Pauciarticular JRA—about 50% of children with JRA have this form.
    • Affects four or fewer joints, usually involving large joints like knees, ankles, wrists, and elbows.
    • Joints are generally affected in an asymmetrical pattern (meaning if a joint on the left side is affected, the opposite joint on the right side is not).
    • Children with this disorder may also develop uveitis, a potentially serious eye condition.
  • Polyarticular JRA—about 40% of children with JRA have this form.
    • Affects five or more joints, usually involving the small joints of the fingers and hands, as well as weight-bearing joints (knees, hips, ankles, and feet).
    • Joints are generally affected in a symmetrical pattern (meaning if a joint on one side is affected, its counterpart on the opposite side is also affected).
  • Systemic Onset JRA (also called Still’s disease)—it is the least common type of JRA, affecting only about 10% of children with JRA.
    • Presents with fever, rash, and arthritis involving multiple joints.
    • Other organ systems may also be affected.

JRA is a potentially serious condition that requires care from your doctor. The sooner JRA is treated, the more favorable the outcome.

Rheumatoid Arthritis

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© 2008 Nucleus Medical Art, Inc.

Causes

JRA is an autoimmune disease. For unknown reasons possibly influenced by genetics and/or environmental factors, the immune system attacks the tissue that lines the inside of one or more joints, limiting the use of the joint and potentially damaging it.

Juvenile idiopathic (meaning cause is not known) arthritis is a term that some experts now use to distinguish the childhood and adult forms of arthritis. The umbrella term juvenile idiopathic arthritis encompasses more types of autoimmune arthritic-like diseases than JRA, and divides them slightly differently. Therefore, it is important to pay attention to the terminology used when talking to doctors about your child’s disease.

Risk Factors

There are no clear risk factors for JRA. In general:

  • The first subtype of pauciarticular JRA typically affects girls under the age of 7.
  • The other subtype of pauciarticular JRA typically affects boys over the age of 8.
  • Polyarticular JRA usually affects girls more often than boys.
  • Systemic onset JRA affects boys and girls equally.

Symptoms

If your child experiences any of these symptoms, do not assume it is due to JRA. These symptoms may be caused by other, less serious health conditions. If your child experiences any one of them, see your physician.

  • 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
  • Eye inflammation
  • Swollen lymph nodes
  • Growth problems, such as:
    • Growth in affected joints may be too fast or too slow, causing one leg or arm to be longer than the other
    • Joints grow unevenly, off to one side
    • Overall growth may be slowed

Symptoms unique to the three major types of JRA are as follows:

Pauciarticular JRA

The first subtype is associated with eye inflammation. Children often test positive for antinuclear antibodies (ANA). The second subtype involves the lower spine. Children also often develop enthesitis (inflammation at the spots where tendons and ligaments attach to the bones), and may experience eye problems.

Polyarticular JRA

Anemia (low red blood cell count) is a common symptom experienced by both subtypes. The first subtype is characterized by the presence of rheumatoid factor (RF) in the blood, a low-grade fever, and/or nodules (bumps on body parts that receive a lot of pressure, such as elbows). The second subtype is less severe and only involves joint inflammation.

Systemic onset JRA

A high fever and chills, often accompanied by a rash on the thighs and chest, appearing off and on for weeks or months are the first signs of systemic onset JRA. Joint inflammation and pain are common as well. In addition, the heart, lungs, and surrounding tissues may become inflamed. Lymph nodes, liver, and/or spleen may become enlarged.

Often, there are remissions (symptoms are better or disappear) and flare-ups (symptoms become worse).

Diagnosis

There is no one clear diagnostic test for JRA. Rather, the doctor/specialist works to rule out other diseases as the cause of the symptoms. Your doctor will ask about your child’s symptoms and personal and family medical histories. The doctor will then perform a physical exam. Once your child's physician suspects JRA or makes this diagnosis, your child may be referred to a pediatric rheumatologist. Tests may include the following:

  • Imaging techniques (such as x-rays)
  • Laboratory tests on blood, urine, and/or joint fluid, to:
  • Ophthalmologic examinations—to detect inflammatory problems of various parts of the eye

Treatment

Talk with your doctor about the best treatment plan for your child. Treatment aims to preserve a high level of physical and social functioning and maintain a good quality of life by controlling inflammation, relieving pain, preventing or controlling joint damage, and maximizing joint function. Treatment options include the following:

Medication

Several types of medication are available to treat JRA:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs)—Examples include ibuprofen, naproxen, diclofenac, or tolmetin.
  • Disease-modifying antirheumatic drugs (DMARDs)—These medications slow the progression of the disease; methotrexate is an example.
  • Corticosteroids—Patients are given these either intravenously (directly into the vein) or by mouth.
  • Immune response modulators—Examples include TNF inhibitors, which are considered in refractory cases.

Physical Therapy

Exercises are performed to maintain muscle tone and preserve and recover the range of motion of the joints. Physical activity in normal daily life, such as non-contact sports and recreational activities, are encouraged along with physical therapy to keep muscles strong and joints mobile. Children are also able to develop confidence in their physical abilities through such physical pursuits.

Maintenance Devices

Splints and other devices are worn to maintain normal bone and joint growth, and prevent flexion contractures (permanently bent joints).

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:

BC Health Guide
http://www.bchealthguide.org

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

References:

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.

JAMA Patient Page. Juvenile idiopathic arthritis. JAMA . 2005;294:1722. Available at: http://jama.ama-assn.org/cgi/content/full/294/13/1722 . Accessed June 21, 2007.

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.



Last reviewed Janaury 2008 by Jill D. Landis, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.


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