A patient diagnosed with pauciarticular juvenile rheumatoid arthritis after a mechanical prosthetic valve replacement due to aortic regurgitation.

Valvular heart disease is a rare complication of juvenile rheumatoid arthritis (JRA), with most cases associated with polyarticular JRA. The aortic valve is most commonly affected, and valvular involvement occurs months or years after the onset of JRA. Reported cases of valvular heart disease in patients with JRA in a pauciarticular pattern are rare. We report a case of severe aortic insufficiency in a 12-year-old boy who underwent aortic valve replacement before diagnosis of JRA with a pauciarticular pattern.