title: Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome and coronary artery dilatation misdiagnosed as Kawasaki disease.
abstract: 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.
authors.affiliation: Division of Pediatric Rheumatology, İstanbul Kanuni Sultan Süleyman Training and Research Hospital, İstanbul, Turkey. keskindemirci@hotmail.com.
authors.forename: Gonca
authors.initials: G
authors.lastname: Keskindemirci
authors.forename: Nuray
authors.initials: N
authors.lastname: Aktay Ayaz
authors.forename: Neslihan
authors.initials: N
authors.lastname: Melikoğlu
authors.forename: Helen
authors.initials: H
authors.lastname: Bornaun
authors.forename: Çiğdem
authors.initials: Ç
authors.lastname: Aydoğmuş
authors.forename: Esin
authors.initials: E
authors.lastname: Aldemir
authors.forename: Gönül
authors.initials: G
authors.lastname: Aydoğan
mesh_terms: D001171:Arthritis, Juvenile / Q000150:complications / Q000175:diagnosis*; D002675:Child, Preschool; D003324:Coronary Artery Disease / Q000150:complications*; D003331:Coronary Vessels / Q000473:pathology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D004108:Dilatation, Pathologic; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D055501:Macrophage Activation Syndrome / Q000150:complications*; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis*