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Lyme arthritis and juvenile rheumatoid arthritis (JRA) share a number of clinical features. Our study was performed in order to determine the prevalence of antibody to Borrelia burgdorferi in 50 children with JRA who reside in a nonendemic area. Three patients were weakly reactive and one patient was reactive when tested using an enzyme immunoassay to detect serum antibody to B. burgdorferi. No patient, however, had definitive serologic evidence of B. burgdorferi infection by Western blot analysis. We conclude that the prevalence of antibody to B. burgdorferi is very low in children with JRA who reside in a nonendemic area.