Juvenile rheumatoid arthritis.

Abstract

1. Jane G. Schaller, MD* 1. 2. *David and Leona Karp Professor of Pediatrics; Chair, Department of Pediatrics, Tufts University School of Medicine, Boston, MA. 1. There are several recognizable subgroups of juvenile rheumatoid arthritis (JRA): systemic-onset, polyarticular, pauciarticular, and late childhood onset. 2. Rheumatoid factor usually is negative in JRA. 3. Systemic-onset JRA is characterized by high intermittent fevers and other extra-articular manifestations: lymphadenopathy, rash, pleuritis, and pericarditis. 4. Iridocyclitis is absent in acute-onset disease but common in patients who have pauciarticular disease. 5. Nonsteroidal anti-inflammatory drugs (NSAIDs) remain the front-line and important agents in the therapy of patients who have JRA. 6. Controlled studies show that d-penicillamine, hydroxychloroquine, and oral gold are no more effective than placebo in the treatment of JRA. Juvenile rheumatoid arthritis (JRA) is a term used to describe the condition of chronic synovitis in children of which there are several distinct subgroups. Chronic synovitis in children was first described well in the English literature in 1897 by George Frederick Still, an English pediatrician and pathologist, who reported on 23 children who had chronic arthritis and came to his attention while he was training at the Hospital for Sick Children, Great Ormond Street. Still postulated that several separate conditions were responsible for chronic arthritis in children. Little subsequent work in this area was pursued until the late 1940s. Most modern observers have come to agree with Still that chronic arthritis in children encompasses several distinct disease subgroups (Table 1⇓ ). The relationship of JRA to adult rheumatoid arthritis was uncertain for many years. Now it seems clear that the classic adult-type rheumatoid arthritis does occur in children but is quite rare, the JRA subgroups seronegative 1 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⇓), but …