면역글로불린 저항성 가와사키병의 인플릭시맵 치료 현황에 관한 한국 다기관 연구


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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

(구연):
Release Date : 2017. 10. 27(금)
Gyu Hur1, Min Seob Song1, Se Jung Sohn 2, Young Mi Hong2, Hyung Doo Lee3, Gi Bum Kim4, Hwa Jin Cho5, Kyung Lim Yoon6, Chan Uhng Joo7 , Chul Ho Kim8
Inje University Haeundae Paik Hospital Department of Pediatrics1
Ewha Womans University Mokdong Hospital Department of Pediatrics2
Pusan National University Children's Hospital Department of Pediatrics3
Seoul National Univesity Children's Hospital Department of Pediatrics4
Chonnam National University Hospital Department of Pediatrics5
Kyunghee University Kyunghee Medical Center Department of Pediatrics6
Chonbuk National University Hospital Department of Pediatrics7
Inje University Busan Paik Hospital Department of Pediatrics8
허규1, 송민섭1, 손세정2, 홍영미2, 이형두3, 김기범4, 조화진5, 윤경림6, 주찬웅7 , 김철호8
인제대학교 해운대백병원 소아청소년과1
이화여자대학교 의과대학부속 목동병원 소아청소년과2
부산대학교 어린이병원 소아청소년과3
서울대학교 어린이병원 소아청소년과4
전남대학교병원 소아청소년과5
경희대학교병원 소아청소년과6
전북대학교병원 소아청소년과7
인제대학교 부산백병원 소아청소년과8

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.

Keywords: Mucocutaneous lymph node syndrome, Intravenous immunoglobulin, infliximab