Skip to main content
Log in

Opinion statement

Kawasaki disease is an acute vasculitis of childhood, complicated by coronary artery aneurysms in up to 25% of affected children. High-dose intravenous immunoglobulin (IVIG) administered in the acute phase of Kawasaki disease leads to a fivefold reduction in the prevalence of coronary aneurysms. When fever persists, a second IVIG infusion should be administered. Rescue therapies for IVIG-resistant Kawasaki disease include pulsed-dose methylprednisolone or other steroid regimens, as well as infliximab, a tumor necrosis factor-α antagonist. Aspirin is used initially in an anti-inflammatory dosage, then at a low dosage until approximately 6 weeks after illness onset. Patients with coronary aneurysms require chronic antithrombotic therapy. For small aneurysms, aspirin alone is sufficient. For larger aneurysms, agents added to aspirin include clopidogrel and, for giant aneurysms, warfarin or low molecular weight heparin. Long-term management is tailored to the degree of coronary artery involvement. Evidence-based data to guide indications for transcatheter and surgical intervention are limited.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References and Recommended Reading

  1. Kawasaki T: Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children [in Japanese]. Jpn J Allergy 1967, 116:178–222.

    Google Scholar 

  2. Melish ME, Hicks RM, Larson EJ: Mucocutaneous lymph node syndrome in the United States. Am J Dis Child 1976, 130:599–607.

    PubMed  CAS  Google Scholar 

  3. Taubert KA, Rowley AH, Shulman ST: Nationwide survey of Kawasaki disease and acute rheumatic fever. J Pediatr 1991, 119:279–282.

    Article  PubMed  CAS  Google Scholar 

  4. Kato H, Sugimura T, Akagi T, et al.: Long-term consequences of Kawasaki disease: a 10-to 21-year follow-up study of 594 patients. Circulation 1996, 94:1379–1385.

    PubMed  CAS  Google Scholar 

  5. Dajani AS, Taubert KA, Gerber MA, et al.: Diagnosis and therapy of Kawasaki disease in children. Circulation 1993, 87:1776–1780.

    PubMed  CAS  Google Scholar 

  6. Newburger JW, Takahashi M, Gerber MA, et al.: 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 2004, 110:2747–2771.

    Article  PubMed  Google Scholar 

  7. Kavey RE, Allada V, Daniels SR, et al.: Cardiovascular risk reduction in high-risk pediatric patients: a scientific statement from the American Heart Association Expert Panel on Population and Prevention Science; the Councils on Cardiovascular Disease in the Young, Epidemiology and Prevention, Nutrition, Physical Activity and Metabolism, High Blood Pressure Research, Cardiovascular Nursing, and the Kidney in Heart Disease; and the Interdisciplinary Working Group on Quality of Care and Outcomes Research: endorsed by the American Academy of Pediatrics. Circulation 2006, 114:2710–2738.

    Article  PubMed  Google Scholar 

  8. Graham TP Jr, Driscoll DJ, Gersony WM, et al.: Task Force 2: congenital heart disease. J Am Coll Cardiol 2005, 45:1326–1333.

    Article  PubMed  Google Scholar 

  9. Independence Blue Cross: ePocrates* Rx Formulary—Clinical and Formulary References. Available at http://www.ibx.com/providers/drug_formulary/epocrates/index.html. Accessed December 23, 2006.

  10. Micromedex. Available at http://www.micromedex.com. Accessed December 23, 2006.

  11. Marasini M, Pongiglione G, Gazzolo D, et al.: Late intravenous gamma globulin treatment in infants and children with Kawasaki disease and coronary artery abnormalities. Am J Cardiol 1991, 68:796–797.

    Article  PubMed  CAS  Google Scholar 

  12. Terai M, Shulman ST: Prevalence of coronary artery abnormalities in Kawasaki disease is highly dependent on gamma globulin dose but independent of salicylate dose. J Pediatr 1997, 131:888–893.

    Article  PubMed  CAS  Google Scholar 

  13. Klassen TP, Rowe PC, Gafni A: Economic evaluation of intravenous immune globulin therapy for Kawasaki syndrome. J Pediatr 1993, 122:538–542.

    Article  PubMed  CAS  Google Scholar 

  14. Arj-ong S, Chotivitayatarakorn P, Lertsapcharoen P, et al.: A cost-benefit analysis of intravenous immunoglobulin treatment in children with Kawasaki disease. J Med Assoc Thai 2003, 86(suppl 2):S179–S188.

    PubMed  Google Scholar 

  15. Catella-Lawson F, Reilly MP, Kapoor SC, et al.: Cyclooxygenase inhibitors and the antiplatelet effects of aspirin. N Engl J Med 2001, 345:1809–1817.

    Article  PubMed  CAS  Google Scholar 

  16. Lee JH, Hung HY, Huang FY: Kawasaki disease with Reye syndrome: report of one case. Acta Paediatrica Sinica 1992, 33:67–71.

    PubMed  CAS  Google Scholar 

  17. Takahashi M, Mason W, Thomas D, Sinatra F: Reye syndrome following Kawasaki syndrome confired by liver histopathology. In Kawasaki Disease. Edited by Kato H. New York: Elsevier Science; 1995:436–444.

    Google Scholar 

  18. Dajani AS, Taubert KA, Takahashi M, et al.: Guidelines for long-term management of patients with Kawasaki disease. Report from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 1994, 89:916–922.

    PubMed  CAS  Google Scholar 

  19. Durongpisitkul K, Gururaj VJ, Park JM, Martin CF: The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin treatment. Pediatrics 1995, 96:1057–1061.

    PubMed  CAS  Google Scholar 

  20. Sundel RP, Baker AL, Fulton DR, Newburger JW: Corticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial. J Pediatr 2003, 142:611–616.

    Article  PubMed  CAS  Google Scholar 

  21. Okada Y, Shinohara M, Kobayashi T, et al.: Effect of corticosteroids in addition to intravenous gamma globulin therapy on serum cytokine levels in the acute phase of Kawasaki disease in children. J Pediatr 2003, 143:363–367.

    Article  PubMed  CAS  Google Scholar 

  22. Shinohara M, Sone K, Tomomasa T, Morikawa A: Corticosteroids in the treatment of the acute phase of Kawasaki disease. J Pediatr 1999, 135:465–469.

    Article  PubMed  CAS  Google Scholar 

  23. Nonaka Z, Maekawa K, Okabe T, Eto Y, Kubo M: Randomized controlled study of intravenous prednisolone and gamma globulin treatment in 100 cases with Kawasaki disease. In Kawasaki Disease. Edited by Kato H. New York: Elsevier Science; 1995:328–331.

    Google Scholar 

  24. Kijima Y, Kamiya T, Suzuki A, et al.: A trial procedure to prevent aneurysm formation of the coronary arteries by steroid pulse therapy in Kawasaki disease. Jpn Circ J 1982, 46:1239–1242.

    PubMed  CAS  Google Scholar 

  25. Inoue Y, Okada Y, Shinohara M, et al.: A multicenter prospective randomized trial of corticosteroids in primary therapy for Kawasaki disease: clinical course and coronary artery outcome. J Pediatr 2006, 149:336–341.

    Article  PubMed  CAS  Google Scholar 

  26. Wooditch AC, Aronoff SC: Effect of initial corticosteroid therapy on coronary artery aneurysm formation in Kawasaki disease: a meta-analysis of 862 children. Pediatrics 2005, 116:989–995.

    Article  PubMed  Google Scholar 

  27. Newburger JW, Sleeper LA, McCrindle BW, et al.: Randomized trial of pulse steroid therapy for primary treatment of Kawasaki disease. N Engl J Med 2006, in press.

  28. Shulman ST: Is there a role for corticosteroids in Kawasaki disease? J Pediatr 2003, 142:601–603.

    Article  PubMed  Google Scholar 

  29. Wright DA, Newburger JW, Baker A, Sundel RP: Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids. J Pediatr 1996, 128:146–149.

    Article  PubMed  CAS  Google Scholar 

  30. Dale RC, Saleem MA, Daw S, Dillon MJ: Treatment of severe complicated Kawasaki disease with oral prednisolone and aspirin. J Pediatr 2000, 137:723–726.

    Article  PubMed  CAS  Google Scholar 

  31. Wallace CA, French JW, Kahn SJ, Sherry DD: Initial intravenous gammaglobulin treatment failure in Kawasaki disease. Pediatr 2000, 105:E78.

    Article  CAS  Google Scholar 

  32. Hashino K, Ishii M, Iemura M, et al.: Re-treatment for immune globulin-resistant Kawasaki disease: a comparative study of additional immune globulin and steroid pulse therapy. Pediatr Int 2001, 43:211–217.

    Article  PubMed  CAS  Google Scholar 

  33. Weiss JE, Eberhard BA, Chowdhury D, Gottlieb BS: Infliximab as a novel therapy for refractory Kawasaki disease. J Rheumatol 2004, 31:808–810.

    PubMed  Google Scholar 

  34. Burns JC, Mason WH, Hauger SB, et al.: Infliximab treatment for refractory Kawasaki syndrome. J Pediatr 2005, 146:662–667.

    Article  PubMed  CAS  Google Scholar 

  35. Finkelstein Y, Nurmohamed L, Avner M, et al.: Clopidogrel use in children. J Pediatr 2005, 147:657–661.

    Article  PubMed  CAS  Google Scholar 

  36. Li JS, Berezny KY, Yow E, et al.: Optimal dose of clopidogrel for platelet inhibition in children: primary results of the PICOLO trial [abstract]. Circulation 2006, 114:II450.

    Google Scholar 

  37. Williams RV, Wilke VM, Tani LY, Minich LL: Does Abciximab enhance regression of coronary aneurysms resulting from Kawasaki disease? Pediatr 2002, 109:E4.

    Article  Google Scholar 

  38. Ishii M, Ueno T, Akagi T, et al.: Guidelines for catheter intervention in coronary artery lesion in Kawasaki disease. Pediatr Int 2001, 43:558–562.

    Article  PubMed  CAS  Google Scholar 

  39. Ishii M, Ueno T, Ikeda H, et al.: Sequential follow-up results of catheter intervention for coronary artery lesions after Kawasaki disease: quantitative coronary artery angiography and intravascular ultrasound imaging study. Circulation 2002, 105:3004–3010.

    Article  PubMed  Google Scholar 

  40. Tsuda E, Kitamura S: National survey of coronary artery bypass grafting for coronary stenosis caused by Kawasaki disease in Japan. Circulation 2004, 110:II61–II66.

    Article  PubMed  Google Scholar 

  41. Kitamura S: The role of coronary bypass operation on children with Kawasaki disease. Coron Artery Dis 2002, 13:437–447.

    Article  PubMed  Google Scholar 

  42. Kitamura S, Kameda Y, Seki T, et al.: Long-term outcome of myocardial revascularization in patients with Kawasaki coronary artery disease. J Thorac Cardiovasc Surg 1994, 107:663–674.

    PubMed  CAS  Google Scholar 

  43. Guidelines for treatment and management of cardiovascular in Kawasaki disease. Subcommittee of Cardiovascular Sequelae, Subcommittee of Surgical Treatment, Kawasaki Disease Research Committee [no authors listed]. Heart Vessels 1987, 3:50–54.

  44. Checchia PA, Pahl E, Shaddy RE, Shulman ST: Cardiac transplantation for Kawasaki disease. Pediatrics 1997, 100:695–699.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Newburger, J.W., Fulton, D.R. Kawasaki disease. Curr Treat Options Cardio Med 9, 148–158 (2007). https://doi.org/10.1007/s11936-007-0008-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11936-007-0008-3

Keywords

Navigation