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A case of life-threatening valvulitis mimicking infective endocarditis in systemic juvenile idiopathic arthritis

Published online by Cambridge University Press:  15 April 2020

Kyung Jin Oh
Department of Pediatrics, Seoul National University Bundang Hospital, Seongnam, Korea
Hye Won Kwon*
Department of Pediatrics, Seoul National University Childrenʼs Hospital, Seoul, Korea
Sungkyu Cho
Department of Thoracic and Cardiovascular Surgery, Seoul National University Hospital, Seoul, Korea
Jeong Wook Seo
Department of Pathology, Seoul National University Hospital, Seoul, Korea
Author for correspondence: H. W. Kwon, Department of Pediatrics, Seoul National University Childrenʼs Hospital, Seoul National University College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul03080, Korea. Tel: +82 2 2072 4306; Fax: +82 2 747 2471. E-mail:


Differential diagnosis of an intracardiac mass is difficult when detected only by echocardiography before a biopsy is completed. However, treatment cannot be postponed until the biopsy results are obtained. We report the case of a 12-year-old girl who presented with an intracardiac mass in the mitral valve mimicking infective endocarditis and severe mitral regurgitation. The mass was finally diagnosed as valvulitis associated with systemic juvenile idiopathic arthritis, which was complicated with macrophage activation syndrome. After careful exclusion of acute infectious disease, we started steroid pulse therapy and administered tocilizumab to treat the cytokine storm before performing the surgery. Finally, we performed mass excision and mitral valve replacement after immunosuppressant therapy.

Brief Report
© The Author(s) 2020. Published by Cambridge University Press

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