Korean Circ J.  2016 Jul;46(4):542-549. 10.4070/kcj.2016.46.4.542.

Non-Responders to Intravenous Immunoglobulin and Coronary Artery Dilatation in Kawasaki Disease: Predictive Parameters in Korean Children

• Kim BY
• Kim D
• Kim YH
• Ryoo E
• Sun YH
• Jeon IS
• Jung MJ
• Cho HK
• Tchah H
• Choi DY
• Kim NY

• KMID: 2344430
• DOI: http://doi.org/10.4070/kcj.2016.46.4.542

Abstract

BACKGROUND AND OBJECTIVES
In Kawasaki disease (KD), high dose intravenous immunoglobulin (IVIG) significantly lowers the coronary complications. However, some patients either do not respond to initial therapy or develop coronary complications. We aimed to identify the predictive factors for unresponsiveness to initial IVIG therapy and coronary artery dilatation (CAD; defined by Z-score≥2.5) in the acute phase and convalescent phase.
SUBJECTS AND METHODS
A retrospective review was conducted of 703 patients with KD, admitted to Gachon University Gil Medical Center between January 2005 and June 2013. The patients were divided into two groups-IVIG responders vs. non-responders-based on the IVIG treatments, and presence of fever after treatment. Further, these groups were divided into two subgroups based on their CAD.
RESULTS
Among the 703 patients with KD, the rate of non-responders to initial IVIG was 16.8%. Serum total bilirubin, platelet count, and neutrophil proportion were independent predictive parameters of unresponsiveness (p<0.05). CAD was found in 234 patients (33.3%) in the acute phase, and in 32 patients (4.6%) in the convalescent phase. Male gender, fever duration, serum C-reactive protein, and white blood cell count were related to CAD (p<0.05). CAD was detected more frequently in non-responders than in the responders (47.5% vs. 31.5%, p=0.001). Kobayashi, Egami, and Sano scoring systems applied to our study population reflected low sensitivities (28.0-33.9%).
CONCLUSION
Several independent parameters were related to unresponsiveness to the initial IVIG or CAD. These parameters might be helpful in establishing more focused and careful monitoring of high-risk KD patients in Korea.

Keyword

Kawasaki disease; Intravenous immunoglobulins; Coronary arteries; Children

MeSH Terms

Bilirubin
C-Reactive Protein
Child*
Coronary Vessels*
Dilatation*
Fever
Humans
Immunoglobulins*
Immunoglobulins, Intravenous
Korea
Leukocyte Count
Male
Mucocutaneous Lymph Node Syndrome*
Neutrophils
Platelet Count
Retrospective Studies
Bilirubin
C-Reactive Protein
Immunoglobulins
Immunoglobulins, Intravenous

Figure

• 

  Fig. 1 Flowchart of enrollment and classification of the subjects. KD: Kawasaki disease, IVIG: intravenous immunoglobulin, CAD: coronary artery dilatation.

• 

  Fig. 2 Mean values of maximum coronary artery Z-scores in IVIG responders and nonresponders. *Analyzed by an unpaired t-test. IVIG: intravenous immunoglobulin.

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  Fig. 3 Frequency of CAD (Z-score≥2.5) in IVIG responders and nonresponders. *Analyzed by a Chi-square test. CAD: coronary artery dilatation, IVIG: intravenous immunoglobulin.

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  Fig. 4 Severity of CAD in IVIG responders and nonresponders. *Analyzed by a Chi-square test with linear by linear association. No dilatation (Z<2.5) in 463 (65.9%), small dilatation (2.5≤Z<5) in 228 (32.4%), large dilatation (5≤Z<10) in 10 (1.4%), and giant dilatation (Z≥10) in 2 (0.3%). CAD: coronary artery dilatation, IVIG: intravenous immunoglobulin.

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