The Turkish Journal of Pediatrics 2015 , Vol 57 , Num 5
Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease
Division of 1Pediatric Rheumatology, 3Pediatric Cardiology, 4Pediatric Immunology, and 5Pediatric Hematology, İstanbul Kanuni Sultan Süleyman Training and Research Hospital and 2Division of Pediatric Cardiology, Mehmet Akif Ersoy Cardiothoracic Surgery Hospital, İstanbul, Turkey. E-mail: keskindemirci@hotmail.com Received: 21 October 2014, Revised: 31 December 2014, Accepted: 11 February 2015 Keskindemirci G, Aktay-Ayaz N, Melikoğlu N, Bornaun H, Aydoğmuş Ç, Aldemir E, Aydoğan G. Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease. Turk J Pediatr 2015; 57: 518-521.

Systemic onset juvenile idiopathic arthritis (SoJIA) is characterized by arthritis, fever and visceral organ involvement including hepatosplenomegaly, lympadenopathy and serositis. This is a case of SoJIA misdiagnosed as Kawasaki disease (KD) and developed machrophage activation syndrome (MAS) secondary to Ebstein-Barr virus (EBV) infection. It is presented to point out the conditions that may come along. First of all, SoJIA should be kept in mind while making the differential diagnosis of coronary arterial ectasias and dilatations usually seen in vasculitic diseases like KD. Second, as a very fatal complication MAS should always be considered while following a patient with the diagnosis of SoJIA. Infections like EBV may be the potential triggers for development of MAS especially in immunesupressed patients. Keywords : EBV infection, MAS, SoJIA.

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