[canakinumab治疗全身性幼年特发性关节炎患儿的难治性巨噬细胞激活综合征]。

Zhonghua er ke za zhi = Chinese journal of pediatrics Pub Date : 2023-09-02 DOI:10.3760/cma.j.cn112140-20230220-00114

X N Zhu, T Y He, S L Luo, Y Y Huang, Y B Xu, S L Liu, J Yang

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[Refractory macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis treated with canakinumab].

患儿　男，3岁7月龄，以发热、关节肿痛为首发表现，外周血白细胞及超敏C反应蛋白增高，病程中患儿血小板下降、铁蛋白升高、纤维蛋白原降低、NK细胞活性下降以及可溶性白细胞介素2受体升高，诊断幼年特发性关节炎全身型合并巨噬细胞活化综合征（MAS）。糖皮质激素冲击并加用卢可替尼口服，启用94噬血方案及2次挽救方案治疗后效果欠佳，患儿仍反复发热，铁蛋白持续升高，诊断难治性MAS。加用卡那单抗并定期维持治疗，患儿5岁，糖皮质激素已停用，疾病持续缓解。.

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Zhonghua er ke za zhi = Chinese journal of pediatrics

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