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.

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:

anemia

iridocyclitis

pericarditis

pleuritis

myocarditis

Tests include:

CBC

ESR

ANA

RA

factor

HLA antigens

immunoelectrophoresis - serum

synovial fluid analysis

X-ray

of a joint

X-ray of the chest

ECG

slit-lamp exam

of the eyes

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.