Juvenile rheumatoid arthritis (JRA) is a condition of chronic synovitis in children of which there are several distinct
subgroups (Table 1). The classic adult-type rheumatoid arthritis does occur in children but is quite rare, the JRA subgroups
seronegative* polyarthritis and systemic-onset disease also occur in adults but not frequently, the pauciarthritis of early
childhood has not been recognized in adults, and the pauciarthritis of older children usually is classified as
spondyloarthropathy in adults. The nomenclature for chronic childhood arthritis remains confusing (Table 2).
The true incidence of JRA is not known. Based on the estimate that approximately 5% of all cases of chronic so-called
"rheumatoid" arthritis begin during childhood, there would be about 250,000 children who have JRA in the United States
today. JRA certainly is not a rarity among the chronic diseases affecting children. There are no good studies of the
comparative incidence of JRA in countries worldwide. Most forms of JRA are known to occur in countries where data are
available; pauciarthritis of older children, related to spondyloarthropathy, is more common in populations such as the native
Americans of western Canada, who have a high prevalence of HLA-B27, a human leukocyte antigen that is associated with
No causes or risk factors have been identified for JRA. Antecedent causes that frequently are mentioned include
infectious agents, immunologic abnormalities of the host, physical trauma to joints, psychological trauma to the child, and
allergy or reactions to environmental agents. Some forms of JRA appear to have a familial occurrence, particularly older
onset pauciarthritis, which often occurs in families whose members have ankylosing spondylitis or other
spondyloarthropathies associated with HLA-B27. Familial occurrences of early childhood pauciarthritis and of seropositive
arthritis also can be found, although not commonly, which is not surprising because both of these forms of arthritis have
associations with genetically determined HLA types. There is no evidence that JRA is transmissible. The various subgroups
of JRA appear to differ in gender, age at onset, types of complications, and prognosis, although the epidemiologic
significance of these observations is unknown (Table 1).
The synovial tissue, or lining tissue of the joint, is the target for inflammation in JRA. Early results of synovial inflammation
include hypertrophy of synovial tissue and secretion of increased amounts of joint fluid. Affected joints are swollen, limited in
motion, stiff, painful, warm, and occasionally erythematous. Two or more of these objective signs are necessary to warrant
the designation of arthritis (Table 3). Affected joints in JRA generally are marked by swelling, stiffness, and loss of motion;
excruciating pain, bright erythema, or dramatic heat are suggestive of some other cause of synovitis, such as septic arthritis
or acute rheumatic fever. The joint fluid in JRA contains increased numbers of cells, predominantly polymorphonuclear
leukocytes; cell counts may range between 10,000 and 100,000/mm3, and synovial fluid glucose levels may be decreased.
Slight male preponderance
Seronegativity: RF* and
Severe arthritis in 25%
small and large joints
Early or late childhood
ANA in 25%
of small and large joints
Late childhood onset
ANA in 50% to 75%
Hips/sacroiliac joints spared
Early childhood onset
ANA in 60%, RF-negative
Chronic iridocyclitis in 30%
The etiologic agent or agents for the synovitis in JRA are unknown. The synovitis is characterized by its chronicity.
Immunologic mechanisms such as immune complex disease may perpetuate the synovitis, but full explanations for the
chronicity of synovial inflammation remain to be found. If synovitis persists long enough in an individual joint, structures of
the joint may be damaged. Because articular cartilage does not regenerate well, this destruction may be permanent.
Fortunately, many children who have JRA seem to withstand prolonged periods of synovitis without incurring permanent
joint destruction. The destructive mechanisms of rheumatoid synovium are not fully known. It is of interest to compare JRA
synovitis with the synovitis of other types of arthritis. The synovitis of staphylococcal arthritis is extremely aggressive and
will cause joint destruction within several days of onset, and the synovitis of classic adult rheumatoid arthritis often results in
joint destruction within 1 year of disease onset. In contrast, the synovitis of the seronegative forms of JRA may not destroy
joint structures even after years of activity. Such differences suggest distinctions either in the nature of the synovitis or of
JRA may involve other organ systems in addition to the joints, and patterns of organ involvement differ in various
subgroups. Possible extraarticular manifestations include the iridocyclitis of early childhood pauciarticular disease; the
rheumatoid nodules and rheumatoid vasculitis of seropositive disease; the fevers, rash, polyserositis, hepatosplenomegaly,
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