Published online June 1, 2005
PEDIATRICS Vol. 116 No. 1 July 2005, pp. e89-e93 (doi:10.1542/peds.2004-2190)
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ELECTRONIC ARTICLE

Coronary Artery Dilation Among Patients Presenting With Systemic-Onset Juvenile Idiopathic Arthritis

Bryce A. Binstadt, MD, PhD*,{ddagger}, Jami C. Levine, MD§, Peter A. Nigrovic, MD*,||, Kimberlee Gauvreau, DSci§, Fatma Dedeoglu, MD*, Robert C. Fuhlbrigge, MD, PhD*, Steven N. Weindling, MD, Jane W. Newburger, MD, MPH§ and Robert P. Sundel, MD*

* Rheumatology Program, Division of Immunology
§ Department of Cardiology, Children's Hospital Boston, Boston, Massachusetts
{ddagger} Section on Immunology and Immunogenetics, Joslin Diabetes Center, Boston, Massachusetts
|| Division of Rheumatology, Immunology, and Allergy, Brigham and Women’s Hospital, Boston, Massachusetts
Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire

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.

Key Words: juvenile rheumatoid arthritis • Still's disease • Kawasaki disease • fever

Abbreviations: IVIG, intravenously administered immunoglobulin • KD, Kawasaki disease • SoJIA, systemic-onset juvenile idiopathic arthritis

Accepted Jan 11, 2005.




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