大剂量静脉注射丙种球蛋白无反应性川崎病的临床分析

Clinical analysis of Kawasaki disease patients with non-responsiveness to high-dose gamma-globulin therapy

目的探讨对大剂量静脉注射丙种球蛋白(IVIG)无反应性川崎病的发生率及临床特点,以及再治疗方案的选择。方法回顾性总结2000年1月至2006年12月入院的KD患儿的临床资料,根据对首次大剂量IVIG有无反应分成IVIG敏感组和无反应组,比较两组的临床特点。结果诊断为川崎病并接受IVIG治疗患儿222例,其中IVIG敏感者185例,无反应者37例,发生率16.67%(37/222)。无反应组接受IVIG治疗时间早,发热时间长,住院时间长,白细胞总数、中性粒细胞比值、CRP明显高于敏感组,而血浆白蛋白明显低于敏感组。IVIG无反应组合并冠状动脉病变14例(37.84%),明显高于IVIG敏感组(15.68%)。IVIG无反应组合并噬血细胞综合征2例,多发性冠状动脉瘤及心肌梗死者1例,多脏器功能衰竭死亡1例。对IVIG无反应者的再治疗,给予IVIG追加疗法,甲基泼尼松龙冲击治疗,泼尼松口服治疗。结论IVIG无反应性川崎病较IVIG敏感性川崎病更易发生冠状动脉病变和严重并发症;接受IVIG治疗时间、发热时间、中性粒细胞比值、CRP、血浆白蛋白是IVIG无反应的危险因素。对IVIG无反应性川崎病可以用IVIG追加治疗,无效者选用糖皮质激素。

Objectives To investigate the incidence, clinical characteristics and re-treatment plan of high-dose intravenous immunoglobulin （IVIG） non-responsiveness Kawasaki disease （KD）. Methods Clinical data of KD patients hospitalized from January 2000 to December 2006 were retrospectively reviewed. All KD patients were divided into two groups, responsiveness group and non-responsiveness group, based on their response to the first high dose IVIG therapy. Results Total 222 KD patients treated with high dose IVIG were included. The incidence of non-responsiveness to IVIG therapy is 16.67% （37/222）. Patients of non-responsiveness group had earlier IVIG treatment , longer duration of fever and hospital stay than those of responsiveness group. WBC, ratio of neutrophil granulocyte and C reaction protein of nonresponsiveness group were higher than that of responsiveness group, but plasma-albumin level of non-responsiveness group was obviously lower than that of responsiveness group. The incidence rate of coronary artery lesion was higher in nonresponsiveness group （14/37, 37.84%） than that of responsiveness group （29/185, 15.68%）. In non-responsiveness group, there were two KD patients with hemophagocytic syndrome, one patient with multiple coronary aneurysm and myocardial infarction and one patient with multisystem organ failure. Re-treatment plan consisted of additional IVIG, methylprednisolone pulse therapy or oral prednisone. Conclusions KD patients with IVIG non-responsiveness were vulnerable to coronary artery lesion and severe complications. Time of starting IVIG therapy, duration of fever, neutrophil ratio, level of CRP and plasma albumin were risk factors of IVIG non-responsiveness KD. Additional IVIG and steroid therapy could be chosen to IVIG non-responsiveness KD patients.