Publication Type:

Journal Article


Rheumatology international, Volume 33, Number 4, p.1065-1069 (2013)



Arthritis, case report, child, diagnostic error, Diagnostic Errors, human, Humans, Juvenile Rheumatoid, juvenile rheumatoid arthritis, macrophage activation syndrome, male, mucocutaneous lymph node syndrome, review


Patients with systemic onset juvenile idiopathic arthritis (SoJIA) are rarely known to develop coronary artery dilatation. The American heart association (AHA) statement on evaluation of suspected Kawasaki disease (KD) would lead some SoJIA patients (particularly in the early stages of the disease) to be inaccurately classified as KD. In addition to the institution of inappropriate therapy with IVIG, misdiagnosis as KD can delay definitive treatment for these SoJIA patients who probably have a worse predicted outcome. We present a 6-year-old male patient with SoJIA who was initially classified as incomplete KD. The child developed life-threatening macrophage activation syndrome (MAS). Previous literature regarding coronary dilatation in SoJIA is also reviewed.


cited By (since 1996)0

Cite this Research Publication

S. Kumar, Vaidyanathan, B., Gayathri, S., and Rajam, L., “Systemic onset juvenile idiopathic arthritis with macrophage activation syndrome misdiagnosed as Kawasaki disease: case report and literature review.”, Rheumatology international, vol. 33, pp. 1065-1069, 2013.