川崎病静脉丙种球蛋白耐药临床判断预测及处理

Intravenous immunoglobulin resistance in Kawasaki disease:Clinical judgement,prediction and management

川崎病是儿童时期常见的急性系统性血管炎,在未接受治疗的病例中,约25%的川崎病患者可出现冠状动脉损害。川崎病是发达国家中儿童获得性心脏病的最常见病因。川崎病的首选治疗为静脉注射丙种球蛋白,可显著降低冠状动脉病变的发生率。如经治疗后仍有发热或体温退而复升,称之为丙种球蛋白无反应,此类患儿冠状动脉损害的发生概率升高。因此,有必要及时识别丙种球蛋白治疗无反应的患儿,并给予强化的初始治疗或补救治疗。文章总结了常见的预测丙种球蛋白无反应的因素,包括患者特征、临床及实验室指标(如年龄、性别、治疗时机、血常规、血生化等)。对于无反应型的患儿,补救治疗包括再次给予丙种球蛋白、糖皮质激素、单克隆抗体、环孢素等。

Kawasaki disease is a common acute systemic vaseulitis in childhood. Among those untreated, around 25% patients may develop coronary artery lesions. It has become the most common reason for aequired heart disease in children in developed countries. The standard therapy for Kawasaki disease is intravenous immunoglobulin, which ean significantly reduce prevalenee of coronary artery lesions. If patients have persistence or reoeeurrenee of fever after treatment, which is recognized as intravenous immunogh）bulin resistance, they have an increased risk of developing coronary artery abnormalities. Theretore, it is neeessary to timely reeognize those who might be resistant to intravenous immunoglobulin in order to administrate intensified initial treatment or rescue therapies. The article summarized common predictors of intravenous immunoglobulin resistance, ineluding patients＇ eharaeteristies, elinieal and laboratory indicators, such as age, gender, timing for treatment, routine blood test, blood biochemical assays, etc. Rescue therapies for unre- sponsive patients include additional intravenous immunoglobulin, steroids, infliximab, cyclosporine, etc.