Juvenile rheumatoid arthritis

Definition:

A chronic, inflammatory disease that may cause joint or connective tissue damage. The onset occurs before age 16.

Alternative Names:

Juvenile chronic polyarthritis; JRA; Still's disease

Causes, incidence, and risk factors:

JRA is thought to belong to the collagen classes of disease (those diseases that involve connective tissue).

It is a complicated disease. The primary manifestation is arthritis, but the disease may involve other body systems such as the heart and lining around the heart (pericardium), lungs and lining around the lungs (pleura), eyes, and skin. Systemic arthritis affects 20% of those with juvenile arthritis and includes fever, rash, and enlarged spleen (splenomegaly) in addition to joint inflammation. JRA is generally divided into five broad groups depending on whether a large number of joints are involved or just a few, whether the rheumatoid factor (a blood test) is positive or negative, and whether there is eye involvement or not.

The five categories of JRA may be roughly broken down as follows:

many involved joints and a positive rheumatoid factor
many involved joints and a negative rheumatoid factor
few involved joints and a positive antinuclear antibody
few involved joints and a positive HLA B27 surface antigen
systemic JRA (throughout the body)

The category determination is generally made by a specialist in rheumatology.

The onset of arthritis may be slow or extremely rapid. An early sign of slow onset may be stiffness on arising in the morning. The arthritis of JRA is characterized by swollen painful joints with pain on motion and sometimes to touch. The skin over the joints is generally not red, but it can be. The systemic form of JRA may first appear with high fevers, chills and a rash but without joint pain. In the systemic form, arthritis may develop months after the appearance of the fever.

The two forms of JRA where there are only a few joints involved often have associated eye disease. The most severe form of eye disease, chronic iridocyclitis of JRA, may lead to visual problems or blindness. The milder form of JRA-associated eye disease is acute iridocyclitis, which generally heals without permanent damage.

The cause of juvenile rheumatoid arthritis is unknown. Growth may be affected during periods of active disease. Girls are affected more often than boys. The peak onset occurs between the ages of 2 to 5 years old and between 9 to 12 years old. Risk factors may be a family history of the disease and recent rubella infection or vaccine.

Symptoms:

General symptoms:

joint stiffness on arising in the morning
limited range of motion
slow rate of growth
hot, swollen, painful joints
fever, low grade (with multiple-joint type disease)
fever, high spiking with chills (with systemic form of disease)
rheumatoid rash
rheumatoid nodules (at sites of pressure)

Symptoms of eye involvement:

Other symptoms:

Signs and tests:

Physical examination may show an enlarged liver (hepatomegaly), enlarged spleen (splenomegaly), or swollen lymph nodes (lymphadenopathy). There may also be signs of:

Tests include:

Treatment:

Treatment is aimed at preserving mobility and joint function and supporting the patient and family through a long chronic illness.

Therapeutic medications include:

aspirin
nonsteroidal antiinflammatory agents (NSAIDS)
corticosteroids
topical ophthalmic corticosteroids
mydriatics
gold therapy
chloroquine agents
immune suppressing agents

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 remits 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:

loss of vision or decreased vision
total joint destruction of the major weight-bearing joints
chronic spondyloarthropathy

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.

Review Date: 11/26/2001
Reviewed By: Birgit Kovacs, M.D., Division of Rheumatology, University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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