면역글로불린 저항성 가와사키병의 인플릭시맵 치료 현황에 관한 한국 다기관 연구
Multicenter study on current status of treating intravenous immunoglobulin resistant Kawasaki disease with infliximab in Korea
Abstract
OBJECTIVE: To investigate on current status of the treatment of multicenter hospitalized children with intravenous immunoglobulin (IVIG) resistant Kawasaki disease with infliximab. METHOD: We performed a retrospective study of 103 IVIG resistant KD patients from March 2010 to February 2017. A total of 12 hospitals participated in this survey. By filling in the questionnaire, data including age, gender, laboratory findings, echocardiographic findings, treatment in each hospital were collected. We divided these patients into 5 groups: group 1 received 2nd IVIG treatment and then received intravenous methylprednisolone pulse (IVMP) treatment and finally required infliximab (n=52, 50.5%), group 2 received infliximab after 2nd IVIG treatment (n=24, 23.3%), group 3 received infliximab after 1st IVIG treatment (n=15, 14.6%), group 4 received infliximab after 2nd IVIG + IVMP treatment (n=10, 9.6%), group 5 received 1st IVIG treatment and then received IVMP treatment and finally required infliximab (n=2, 2%). We compared each groups. RESULT: One hundread and three patients (mean: 32 months, 31 females) who received infliximab after 1st IVIG treatment were studied. Overall complete response rate of infliximab treatment was 94/103 (91%). In group 1, the response rate of infliximab was 92.3% (48/52), coronary artery lesion (CAL) occurred in 32 patients (61.5%). In group 2, the response rate of infliximab was 95.9% (23/24), CAL occurred in 2 patients 8.3% (2/24). In group 3, the response rate of infliximab was 73% (11/15), CAL did not occur. In group 4, the response rate of infliximab was 70% (7/10), CAL occurred in 8 patients 80% (8/10). In group 5, the response rate of infliximab was 100% (2/2), CAL occurred in 2 patients 100% (2/2). CONCLUSION: Currently the treatment of IVIG resistant Kawasaki disease diversifies, in which repeated IVIG, IVMP, infliximab are the main choices. This retrospective multicenter study suggests that early treatment of infliximab rather than IVMP after repeated IVIG treatment seems to prevent the CALs. However, further prospective randomized studies may be needed to determine the best treatment options.