Juvenile Rheumatoid Arthritis: A Review

Juvenile rheumatoid arthritis (JRA) is a disease of varying clinical manifestations, although all patients share the common property of arthritis. In following 124 children with JRA at a children's arthritis clinic (mean disease duration, 7.5 years), it was apparent that there were three distinct subgroups of disease distinguished by their clinical manifestations, prognosis for joint disability, types of extra-articular complications, and serologic findings. Systemic disease, characterized by high intermittent fevers and rheumatoid rash as well as other systemic manifestations, occurred in 32 of 124 patients (26%). All patients also had polyarthritis, generally beginning during the first six months of disease. More boys (18) than girls (14) were affected. No patient had iridocyclitis or positive tests for antinuclear antibodies or rheumatoid factor. Systemic symptoms alone were not a cause of permanent morbidity, but eight of 32 patients have incurred severe joint disability. Polyarticular disease unassociated with prominent systemic disease occurred in 46 of 124 patients (37%), predominantly girls (38 girls, eight boys). Multiple joints were involved, characteristically including small hand joints. Ten patients had antinuclear antibodies; six had positive latex agglutination tests for rheumatoid factor. Eight of 46 had severe joint disability. Persistent pauciarticular disease, characterized by arthritis limited to five or fewer joints, occurred in 46 of 124 patients (37%), predominantly girls (34 girls, 12 boys). Large joints were chiefly affected (knees, ankles, elbows); small hand joints were spared. Nine of 46 patients had antinuclear antibodies. No patient has had severe joint disability, but 12 of 46 have had iridocyclitis and ten have suffered some degree of permanent visual damage. Recognition of these disease patterns is useful in the diagnosis and care of children with JRA. Furthermore, these observations suggest that what we now call JRA may in fact be more than a single disease.