静脉注射人丙种球蛋白在儿童风湿性疾病中的应用

Application of intravenous immunoglobulin G in children with rheumatic diseases

静脉注射人丙种球蛋白(IVIG)使用指征主要为抗体缺陷病的替代治疗和大剂量IVIG的免疫调节。由于IVIG的免疫调节作用,其广泛应用于儿童风湿性疾病领域,但多属"超说明书用药"。在儿童风湿性疾病的治疗中,IVIG对于川崎病和原发性免疫性血小板减少症能明确获益,对于干燥综合征、皮肌炎、系统性红斑狼疮等可能获益,而对于Stevens-Johnson综合征、抗中性粒细胞胞质抗体(ANCA)相关性血管炎、系统性硬皮病则仅有一定获益可能。在儿童风湿性疾病的治疗中,IVIG的应用尚需进一步规范。

The indications for the application of intravenous immunoglobulin G(IVIG)mainly include the replacement therapy of antibody deficiency diseases and immunomodulatory therapy with a high dose of IVIG.IVIG is extensively used in children with rheumatic diseases due to its immunomodulatory effects.However,most of them are"off-label drugs".In the treatment of pediatric rheumatic diseases,IVIG can benefit Kawasaki disease and primary immune thrombocytopenia,and may be beneficial in treating Sjögren′s syndrome,dermatomyositis and systemic lupus erythematosus.However,for the patients with Stevens-Johnson syndrome,antineutrophil cyctoplasmic antibody(ANCA)asso-ciated vasculitis and systemic scleroderma,there is not much chance to obtain benefits.In the treatment of rheumatic diseases in children,the application of IVIG needs to be further standardized.