Risk of coronary arterial lesions in immunoglobulin resistant Kawasaki disease


Objective: High-dose intravenous immunoglobulin (IVIG) decreases the risk for development of coronary arterial lesions (CAL) in Kawasaki disease (KD) while patients resistant to IVIG have higher risk. This study is aimed to investigate an additional therapy affects the risk for CAL in IVIG resistant KD. Design: The information from 11,510 children with KD was collected by the nationwide survey (2003-2004) in Japan. The secondary therapeutic strategies for IVIG resistant KD were an additional IVIG dose, steroids or both. The incidence of CAL was compared among 4 groups: G1, children responded to initial IVIG; G2, IVIG resistant patients receiving an additional IVIG dose; G3, IVIG resistant patients receiving additional steroids; G4, IVIG resistant patients receiving an additional IVIG dose plus steroids. CAL was assessed at 30th day of illness using ultrasound echocardiography. Results: 2229 patients (19.4%) were resistant to initial IVIG. Incidence of CAL was significantly lower in children responding to initial IVIG than in IVIG resistant patients (1.9% and 11.0%, respectively; odd’s ratio 6.3). The incidences of CAL in an each additional therapy group were as follows: G2: 6.7%, G3: 9.7%, G4: 22.2%. The risk for CAL was significantly higher in G4 as compared with G2 and G3. Giant aneurysmal formation (GA) was more frequently developed in G3 and G4 consisting of the patients receiving steroids (G1: 0.03%, G2: 0.63%, G3: 4.3%, G4: 3.7%). Conclusion: The incidence of CAL was considerably high in IVIG resistant KD and an additional therapy by steroids may increase the risk for GA.

Share and Cite:

Ogino, H. , Kaneko, K. , Uchiyama, T. , Yoshimura, K. , Teraguchi, M. and Nakamura, Y. (2012) Risk of coronary arterial lesions in immunoglobulin resistant Kawasaki disease. Open Journal of Pediatrics, 2, 38-41. doi: 10.4236/ojped.2012.21005.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Furusho, K., Kamiya, T., Nakano, H., Kiyosawa, N., Shinomiya, K., Hayashidera, T., et al. (1984) High-dose intravenous gammaglobulin for Kawasaki disease. Lancet, 2, 1055-1058. doi:10.1016/S0140-6736(84)91504-6
[2] Newburger, J.W., Takahashi, M., et al. (1986) The treatment of Kawasaki syndrome with intravenous gamma globulin. The New England Journal of Medicine, 315, 341-347. doi:10.1056/NEJM198608073150601
[3] Newburger, J.W., Takahashi, M., Beiser, A.S., et al. (1991) A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. The New England Journal of Medicine, 324, 1633-1639. doi:10.1056/NEJM199106063242305
[4] Burns, J.C., Capparelli, E.V., Brown, J.A., Newburger, J.W. and Glode, M.P. (1988) Intravenous gamma-globulin treatment and retreatment in Kawasaki disease. Pediatric Infectious Disease Journal, 17, 1144-1148. doi:10.1097/00006454-199812000-00009
[5] Durongpisitkul, K., Soongswang, J., Laohaprasitiporn, D., Nana, A., Prachuabmoh, C. and Kangkagate, C. (2003) Immunoglobulin failure and retreatment in Kawasaki disease. Pediatric Cardiology, 24, 145-148. doi:10.1007/s00246-002-0216-2
[6] Egami, K., Muta, H., Ishii, M., et al. (2006) Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease. Journal of Pediatrics, 149, 237-240. doi:10.1016/j.jpeds.2006.03.050
[7] Han, R.K., Silverman, E.D., Newman, A. and McCrindle, B.W. (2000) Management and outcome of persistent or recurrent fever after initial intravenous gamma globulin therapy in acute Kawasaki disease. Archives of Pediatrics & Adolescent Medicine, 154, 694-699.
[8] Wallace, C.A., French, J.W., Kahn, S.J. and Sherry, D.D. (2000) Initial intravenous gamma globulin treatment failure in Kawasaki disease. Pediatrics, 105, e78-e81. doi:10.1542/peds.105.6.e78
[9] Newburger, J.W., Takahashi, M., et al. (2004) Diagnosis, treatment, and long-term management of Kawasaki disease: A statement for health professionals from the committee on rheumatic fever, endocarditis and Kawasaki disease, council on cardiovascular disease in the young, American Heart Association. Circulation, 110, 2747-2771. doi:10.1161/01.CIR.0000145143.19711.78
[10] Wright, D.A., Newburger, J.W., Baker, A. and Sundel, R.P. (1996) Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroidss. Jour- nal of Pediatrics, 128, 146-149. doi:10.1016/S0022-3476(96)70447-X
[11] Okada, M., Nakai, S., Kobayashi, Y., et al. (1997) Effects of intravenous gamma globulin and ulinastatin on patients with Kawasaki disease and predicted giant coronary artery aneurysms. Journal of The Japan Pediatric Society, 101, 1165-1170.
[12] Mori, M., Tomono, N. and Yokota, S. (1995) Coronary arteritis of Kawasaki disease unresponsive to high-dose intravenous gammaglobulin successfully treated with plasmapheresis. Nihon Rinsho, 18, 282-288. doi:10.2177/jsci.18.282
[13] Research Committee on Kawasaki Disease (1984) Report of subcommittee on standardization of diagnostic criteria and reporting of coronary artery lesions in Kawasaki disease. Ministry of Health and Welfare, Tokyo.
[14] Sundel, R.P., Burns, J.C., Baker, A., Beiser, A.S. and Newburger, J.W. (1993) Gamma globulin re-treatment in Kawasaki disease. Journal of Pediatrics, 123, 657-659. doi:10.1016/S0022-3476(05)80972-2
[15] Raman, V., Kim, J., Sharkey, A. and Chatila, T. (2001) Response of refractory Kawasaki disease to pulse steroids and cyclosporin A therapy. Pediatric Infectious Disease Journal, 20, 635-637. doi:10.1097/00006454-200106000-00022
[16] Lee, M.S., An, S.Y., Jang, G.C. and Kim, D.S. (2002) A case of intravenous immunoglobulin-resistant Kawasaki disease treated with methotrexate. Yonsei Medical Journal, 43, 527-532.
[17] Weiss, J.E., Eberhard, B.A., Chowdhury, D. and Gottlieb, B.S. (2004) Infliximab as a novel therapy for refractory Kawasaki disease. Journal of Rheumatology, 31, 808-810.
[18] Burns, J.C., Mason, W.H., et al. (2005) Infliximab treatment for refractory Kawasaki syndrome. Journal of Pediatrics, 146, 662-667. doi:10.1016/j.jpeds.2004.12.022
[19] Kato, H., Koike, S. and Yokoyama, T. (1979) Kawasaki disease: Effect of treatment on coronary artery involvement. Pediatrics, 63, 175-179.
[20] Furukawa, T., Kishiro, M., et al. (2008) Effects of steroid pulse therapy on immunoglobulin-resistant Kawasaki disease. Archives of Disease in Childhood, 93, 142-146. doi:10.1136/adc.2007.126144

Copyright © 2024 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.