Giant aneurysms: A gender-specific complication of Kawasaki disease?
BACKGROUND
Kawasaki disease (KD) is a pediatric vasculitis of unknown origin. Its main complication is the development of coronary artery aneurysms (CAA) with giant CAA at the end of the spectrum.


METHODS
In this cohort study, we evaluated the association between patient characteristics and the development of giant CAA based on z-scores. Multivariable, multinomial logistic regression analysis was used to identify variables associated with giant CAA.


RESULTS
A total of 301 KD patients, comprising 216 patients without enlargement, 45 with small-sized, 19 with medium-sized, and 21 with giant CAA with all echocardiographies at our center were retrospectively included. Remarkably, 95% of patients with giant CAA were boys. In addition to 'no/late intravenous immunoglobulin (IVIG) treatment', 'male gender' (OR 16.23, 95% CI 1.88-140.13), 'age<1 year' (OR 7.49, 95% CI 2.29-24.46), and 'IVIG re-treatment (9.79, 95% CI 2.79-34.37)' were significantly associated with an increased risk of giant CAA, with patients without enlargement as reference. Compared to patients with medium-sized CAA, 'IVIG re-treatment' was significantly associated with giant CAA. The majority of giant CAA continued to increase in size during the first 40 days.


CONCLUSIONS
We identified risk factors associated with an increased risk of giant CAA. The difference in variables between the giant CAA group and the other CAA subgroups suggests a separation between patients with the treatment-resistant giant CAA and the other IVIG-responsive patients, in which gender may be factored as a most relevant genetic trait. The increase in size during the first 2 months indicates the need for repeated echocardiography.
Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands. Electronic address: s.m.dietz@amc.uva.nl.
Sanne M
SM
Dietz
Department of Pediatric Cardiology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Irene M
IM
Kuipers
Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Carline E A
CEA
Tacke
Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Jeffrey C D
JCD
Koole
Department of Clinical Epidemiology, Biostatistics and Bioinformatics, Academic Medical Centre, Amsterdam, The Netherlands.
Barbara A
BA
Hutten
Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Taco W
TW
Kuijpers
D002675:Child, Preschool; D015331:Cohort Studies; D003323:Coronary Aneurysm / Q000000981:diagnostic imaging / Q000188:drug therapy / Q000209:etiology*; D004452:Echocardiography; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007223:Infant; D008297:Male; D009080:Mucocutaneous Lymph Node Syndrome / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D012307:Risk Factors; D012737:Sex Factors
D016756:Immunoglobulins, Intravenous
(Giant) coronary aneurysms; Kawasaki disease; Risk factors