This report describes a 7-week-old infant with incomplete and atypical Kawasaki disease, an acute vasculitis that predominantly affects infants and children. The patient was refractory to 2 doses of intravenous immunoglobulin and to high-dose intravenous methylprednisolone. He became afebrile only after 2 doses of infliximab. His prolonged, recalcitrant course was complicated by the development of peripheral gangrene and giant coronary artery aneurysms. Infants with incomplete and atypical Kawasaki disease are prone to intravenous immunoglobulin treatment failure and are at risk for the development of coronary artery aneurysms. In such patients, we suggest that consideration be given to early aggressive therapy with corticosteroids or infliximab added to intravenous immunoglobulin.
Newburger JW , Takahashi M., Burns JC, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med.1986;315:341-347.
2.
Newburger JW , Takahashi M., Beiser AS, et al. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med.1991;324:1633-1639.
3.
Sundel RP, Burns JC, Baker A., Beiser AS, Newburger JWGamma globulin re-treatment in Kawasaki disease. J Pediatr.1993;123:657-659.
4.
Burns JC, Capparelli EV, Brown JA, Newburger JW, Glode MPIntravenous gamma-globulin treatment and retreatment in Kawasaki disease. Pediatr Infect Dis J.1998;17:1144-1148.
5.
Newburger JW , Takahashi M., Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease. Circulation.2004;l10:2747-2771.
6.
Taubert KA, Rowley AH, Shulman STSeven-year national survey of Kawasaki disease and rheumatic fever. Pediatr Infect Dis J.1994;13:704-708.
7.
Burns JC, Wiggins JW, Toews WH, et al. Clinical spectrum of Kawasaki disease in infants younger than 6 months of age. J Pediatr.1986;109:759-763.
8.
Rosenfeld EA , Corydon KE, Shulman STKawasaki disease in infants less than one year of age. J Pediatr.1995;126:524-529.
9.
Tsuchida S., Yamanaka T., Tsuchida R., Nakamura Y., Yashiro M., Yanagawa H.Epidemiology of infant Kawasaki disease with a report of the youngest neonatal case ever reported in Japan. Acta Paediatr.1996;85:995-997.
10.
Hara T., Mizuno Y., Akeda H., et al. Thrombocytopenia: a complication of Kawasaki disease. Eur J Pediatr.1988;147:51-53.
11.
Tomita S., Chung K., Mas M., Gidding S., Shulman STPeripheral gangrene associated with Kawasaki disease. Clin Infect Dis.1992;14:121-126.
12.
Chang JS, Lin JS, Peng CT, Tsai CHKawasaki disease complicated by peripheral gangrene. Pediatr Cardiol.1999;20:139-142.
Fukunishi M. , Kikkawa M., Hamana K., et al. Prediction of non-responsiveness to intravenous high-dose gamma globulin therapy in patients with Kawasaki disease at onset. J Pediatr.2000;137:172-176.
15.
Durongpisitkul K., Soongswang J., Laohaprasitiporn D., Nana A., Prachuabmoh C., Kangkagate C.Immunoglobulin failure and retreatment in Kawasaki disease. Pediatr Cardiol.2003;24:145-148.
16.
Wright DA, Newburger JW, Baker A., Sundel RPTreatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids. J Pediatr.1996;128:146-149.
17.
Dale RC, Saleem MA, Daw S., Dillon MJTreatment of severe complicated Kawasaki disease with oral prednisolone and aspirin. J Pediatr.2000;137:723-726.
18.
Takeshita S. , Kawamura Y., Nakatani K., Tsujimoto H., Tokutomi T.Standard-dose and short-term corticosteroid therapy in immunoglobulin-resistant Kawasaki disease. Clin Pediatr.2005;44:423-426.
19.
Knight DM, Trinh H., Le J., et al. Construction and initial characterization of a mouse-human chimeric anti-TNF antibody. Mol Immunol.1993;30:1443-1453.
20.
Maury CP, Salo E., Pelkonen P.Elevated circulating tumor necrosis factor-α in patients with Kawasaki disease. J Lab Clin Med.1989;113:651-654.
21.
Matsubara T. , Furukawa S., Yabatu K.Serum levels of tumor necrosis factor, interleukin 2 receptor, and interferon-gamma in Kawasaki disease involved coronary-artery lesions. Clin Immunol Immunopathol. 1990;56:29-36.
22.
Weiss JE, Eberhard A., Chowdhury D., Gotlieb BSInfliximab as a novel therapy for refractory Kawasaki disease. J Rheumatol.2004;31:808-810.
23.
Burns JC, Mason WH, Hauger SB, et al. Infliximab treatment for refractory Kawasaki syndrome. J Pediatr.2005;146:662-667.
24.
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.
25.
Sundel RP, Baker A., Fulton DR, Newburger JWCorticosteroids in the initial treatment of Kawasaki disease: report of a randomized trial. J Pediatr.2003;142:611-616.
26.
Wooditch AS , Aronoff SCEffect of initial corticosteroid therapy on coronary artery aneurysm formation in Kawasaki disease: a meta-analysis of 862 children. Pediatrics. 2005; 116:989-995.
