中国实用儿科杂志

中国实用儿科杂志

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全身型幼年特发性关节炎合并巨噬细胞活化综合征诊断和治疗

  

  1. 浙江大学医学院附属儿童医院风湿免疫科，浙江  杭州  310000
  • 出版日期:2021-01-06 发布日期:2021-02-18

Diagnosis and treatment of systemic juvenile idiopathic arthritis combined with macrophage activation syndrome

  1. Department of Rheumatology Immunology and Allergy， the Children’s Hospital， Zhejiang University School of Medicine， Hangzhou  310000， China
  • Online:2021-01-06 Published:2021-02-18

摘要： 巨噬细胞活化综合征（MAS）是继发于风湿免疫性疾病基础上的噬血细胞性淋巴组织细胞增多症（HLH）， 也是儿科急性危重症， 其中， 以全身型幼年特发性关节炎（sJIA）相关MAS（sJIA-MAS）最为常见， 且具有较高的病死率， 早期诊断、 及时治疗是改善预后的关键。虽然sJIA-MAS和HLH具有相似的临床和实验室特征， 但由于二者的基础疾病不同， 因此诊断标准也有所差异。糖皮质激素和环孢素A是MAS的一线治疗药物， 但仍存在较大挑战，生物制剂靶向药物逐步被提出作为难治性MAS的治疗选择， 甚至作为MAS的一线药物。该文重点阐述sJIA-MAS
的临床和实验室特征、 生物标志物、 分类标准及治疗进展。

关键词: 全身型幼年特发性关节炎, 巨噬细胞活化综合征, 噬血细胞性淋巴组织细胞增多症

Abstract: Macrophage activation syndrome（MAS），a hemophagocytic lymphohistiocytosis（HLH） secondary to rheumatic immune diseases，is a life-threatening complication in children. The most common rheumatic disease associated with MAS is systemic juvenile idiopathic arthritis（sJIA-MAS） and death rate is high. Early diagnosis and timely intervention for sJIA-MAS is critical to improve the prognosis. Although there are similar clinical and laboratory features，classification criteria has a little difference between sJIA-MAS and HLH because of their different basic diseases. Glucocorticoids and cyclosporin A are the first line medications for sJIA-MAS. However，the treatment still remains a major challenge. Biologic agents as targeted medicines are gradually proposed as therapeutic options for refractory MAS，or even as first line medicines. Here we will focus on the clinical and laboratory features，biomarkers，classification criteria and treatment advances of sJIA-MAS.

Key words: systemic juvenile idiopathic arthritis, macrophage activation syndrome, hemophagocytic lymphohistiocytosis