Prevention of large coronary artery lesions caused by Kawasaki disease

Toshimasa Nakada


Appropriate therapy during the acute phase of Kawasaki disease to prevent large coronary artery lesions (CAL) has not been established. The aim of this retrospective study was to investigate the usefulness of initial intravenous immunoglobulin (IVIG) therapy with delayed administration of antiinflammatory drugs (ADs).  A total of 132 pediatric patients who received IVIG therapy with delayed administration of ADs for Kawasaki disease between 2004 and 2014 were enrolled at the Department of Pediatrics, Aomori Prefectural Central Hospital. An initial IVIG regimen of 2 g/kg/day, starting on day 5, was used as first-line therapy when possible. Second-line therapy was additional IVIG therapy, and third-line therapy was an urinastatin infusion or plasma exchange.  All 132 patients received 2 g/kg/day initial IVIG therapy.  Seventy-four patients received aspirin and 58 patients received flurbiprofen after completion of initial IVIG infusion.  Initial IVIG therapy resistance occurred in 31 of 132 patients (23%), and 10 patients (8%) received additional IVIG. One patient received urinastatin and one patient received plasma exchange as third-line therapy.  Before the 30th day, the prevalence of CAL was 2% (2/132); after 30 days, it was 1% (1/132).  The maximal internal CAL diameters were 4.8 mm (Z score = 6.3) among all patients.  Initial single IVIG therapy with delayed administration of ADs may be useful for the prevention of large CAL caused by Kawasaki disease.


Kawasaki disease, intravenous immunoglobulin therapy, coronary artery lesions,

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Gupta M, Noel GJ, Schaefer M Friedman D, Bussel J, Johann-Liang R. 2001. “Cytokine modulation with immune gamma-globulin in peripheral blood of normal children and its implications in Kawasaki disease treatment.” J Clin Immunol 21:193–9

Hokosaki T, Mori M, Nishizawa T et al. 2012. “Long-term efficacy of plasma exchange treatment for refractory Kawasaki disease.” Pediatr Int 54:99–103

Kobayashi T, Saji T, Otani T et al. 2012. “Efficacy of immunoglobulin plus prednisolone for prevention of coronary artery abnormalities in severe Kawasaki disease (RAISE study): a randomized, open-label, blinded-endpoints trial.” Lancet 379:1613–20

Miyamoto T, Ikeda K, Ishii Y, Kobayashi T. 2014. “Rupture of a coronary artery aneurysm in Kawasaki disease: a rare case and review of the literature for the past 15 years.” J Thorac and Cardiovasc Surg 147:e67–9

Mueller F, Knirsch W, Harpes P, Prêtre R, Valsangiacomo Buechel E, Kretschmar O. 2009. “Long-term follow-up of acute changes in coronary artery diameter caused by Kawasaki disease: risk factors for development of stenotic lesions.” Clin Res Cardiol 98:501–7

Muta H, Ishii M, Egami K et al. 2004. “Early intravenous gamma-globulin treatment for Kawasaki disease: the nationwide surveys in Japan.” J Pediatr 144:496–9

Nakada T. 2015. “Effects of anti-inflammatory drugs on intravenous immunoglobulin therapy in the acute phase of Kawasaki disease.” Pediatr Cardiol 36:335–9

Newburger JW, Takahashi M, Beiser AS et al. 1991. “A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome.” N Engl J Med 324:1633–9

Research committee on Kawasaki disease. 1994. “Report of subcommittee on standardization of diagnostic criteria and reporting of coronary artery lesions in Kawasaki disease.” Ministry of Health and Welfare, Tokyo

Takahara T, Yamagami Y, Oonishi S et al. 2014. “Therapeutic strategy for immunoglobulin refractory Kawasaki disease including plasma exchange therapy in 60 patients.” Prog Med 34:1282–7

The Japan Kawasaki disease research committee. 2002. “Diagnostic guidelines of Kawasaki disease.” 5th revised edn, Tokyo

Tremoulet AH, Jain S, Jaggi P et al. 2014. “Infliximab for intensification of primary therapy for Kawasaki disease: a phase 3 randomised, double-blind, placebo–controlled trial.” Lancet 383:1731–38

Tsuda E, Kamiya T, Ono Y, Kimura K, Kurosaki K, Echigo S. 2005. “Incidence of stenotic lesions predicted by acute phase changes in coronary arterial diameter during Kawasaki disease.” Pediatr Cardiol 26:73–9

Uehara R, Belay ED, Maddox RA et al. 2008. “Analysis of potential risk factors associated with nonresponse to initial intravenous immunoglobulin treatment among Kawasaki disease patients in Japan.” Pediatr Infect Dis J 27:155–60

Zorzi AD, Colan SD, Gauvreau K, Baker AL, Sundel RP, Newburger JW. 1998. “Coronary artery dimensions may be misclassified as normal in Kawasaki disease.” J Pediatr 133:254–8

DOI: http://dx.doi.org/10.18103/mra.v0i3.138


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