Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis.
OBJECTIVE
To evaluate coronary artery diameters among patients presenting with systemic-onset juvenile idiopathic arthritis (SoJIA).


METHODS
Fifty cases of SoJIA were reviewed. At the time of initial presentation with fever, 12 patients had echocardiograms that included a complete evaluation of the coronary arteries. A single reviewer measured the diameters of the left main, proximal left anterior descending, and proximal right coronary arteries. Body surface area-adjusted z scores were calculated with respect to a normative population.


RESULTS
Coronary artery dilation (z score: >2) was observed for 5 of the 12 patients with SoJIA who had echocardiograms performed at the time of presentation with fever. No patient developed a coronary artery aneurysm, and all of the coronary artery z scores normalized within 4 months. Only 2 of the 5 patients with coronary artery z scores of >2 fulfilled the clinical criteria for Kawasaki disease, the most commonly recognized cause of coronary artery dilation among children.


CONCLUSIONS
Children presenting with SoJIA may have coronary artery dilation similar to that observed for children with Kawasaki disease. These data suggest that the presence of coronary artery dilation on initial echocardiograms for patients with fever does not exclude the diagnosis of SoJIA.
Binstadt
Bryce A
BA
Rheumatology Program, Division of Immunology, Children's Hospital Boston, Boston, Massachusetts 02115, USA.
Levine
Jami C
JC
Nigrovic
Peter A
PA
Gauvreau
Kimberlee
K
Dedeoglu
Fatma
F
Fuhlbrigge
Robert C
RC
Weindling
Steven N
SN
Newburger
Jane W
JW
Sundel
Robert P
RP
D001171:Arthritis, Juvenile / Q000175:diagnosis / Q000473:pathology*; D002648:Child; D003331:Coronary Vessels / Q000000981:diagnostic imaging / Q000473:pathology*; D003937:Diagnosis, Differential; D004108:Dilatation, Pathologic; D004452:Echocardiography; D005334:Fever; D006801:Humans; D009080:Mucocutaneous Lymph Node Syndrome / Q000175:diagnosis