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.

[1]  Y. Yamashiro,et al.  Effects of steroid pulse therapy on immunoglobulin-resistant Kawasaki disease , 2007, Archives of Disease in Childhood.

[2]  T. Matsuishi,et al.  Prediction of resistance to intravenous immunoglobulin treatment in patients with Kawasaki disease. , 2006, The Journal of pediatrics.

[3]  J. Burns,et al.  Infliximab treatment for refractory Kawasaki syndrome. , 2005, The Journal of pediatrics.

[4]  Walter R Wilson,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 , 2004, Circulation.

[5]  Walter R Wilson,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 , 2004, Pediatrics.

[6]  B. Gottlieb,et al.  Infliximab as a novel therapy for refractory Kawasaki disease. , 2004, The Journal of rheumatology.

[7]  K. Durongpisitkul,et al.  Immunoglobulin Failure and Retreatment in Kawasaki Disease , 2003, Pediatric Cardiology.

[8]  D. S. Kim,et al.  A case of intravenous immunoglobulin-resistant Kawasaki disease treated with methotrexate. , 2002, Yonsei medical journal.

[9]  T. Chatila,et al.  Response of refractory Kawasaki disease to pulse steroid and cyclosporin A therapy. , 2001, The Pediatric infectious disease journal.

[10]  B. McCrindle,et al.  Management and outcome of persistent or recurrent fever after initial intravenous gamma globulin therapy in acute Kawasaki disease. , 2000, Archives of pediatrics & adolescent medicine.

[11]  D. Sherry,et al.  Initial intravenous gammaglobulin treatment failure in Kawasaki disease. , 2000, Pediatrics.

[12]  J. Newburger,et al.  Intravenous gamma-globulin treatment and retreatment in Kawasaki disease. US/Canadian Kawasaki Syndrome Study Group. , 1998, The Pediatric infectious disease journal.

[13]  T. Satoh,et al.  Effects of Intravenous Gamma globulin and Ulinastatin on Patients with Kawasaki Disease and Predicted Giant Coronary Artery Aneurysms , 1997 .

[14]  M. Mori,et al.  [Coronary arteritis of Kawasaki disease unresponsive to high-dose intravenous gammaglobulin successfully treated with plasmapheresis]. , 1995, Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology.

[15]  J. Newburger,et al.  Gamma globulin re-treatment in Kawasaki disease. , 1993, The Journal of pediatrics.

[16]  M Takahashi,et al.  A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. , 1991, The New England journal of medicine.

[17]  J. Newburger,et al.  The treatment of Kawasaki syndrome with intravenous gamma globulin. , 1986 .

[18]  Katsuko Sato,et al.  HIGH-DOSE INTRAVENOUS GAMMAGLOBULIN FOR KAWASAKI DISEASE , 1983, The Lancet.

[19]  S. Koike,et al.  Kawasaki disease: effect of treatment on coronary artery involvement. , 1979, Pediatrics.

[20]  J. Newburger,et al.  Treatment of immune globulin-resistant Kawasaki disease with pulsed doses of corticosteroids. , 1996, The Journal of pediatrics.