巨噬细胞活化综合征(MAS)是一种继发于风湿免疫疾病的噬血细胞性淋巴组织细胞增多症(HLH),是一种罕见的、危及生命的过度炎症性疾病。巨噬细胞活化综合征的典型临床症状包括持续高热、皮疹以及肝和脾的肿大,实验室的检测结果显示患者的血细胞数量减少,伴有高甘油三酯或低纤维蛋白原、高铁蛋白血症和转氨酶水平上升等现象。在全身性幼年特发性关节炎(sJIA)和成人发病斯蒂尔氏病(AOSD)中,巨噬细胞活化综合征的发病率较高,但目前有越来越多的学者对系统性红斑狼疮患者发生巨噬细胞活化综合征进行了报道。巨噬细胞活化综合征病情复杂,进展凶险,若误诊或延误治疗时机,可能造成无法挽回的不良结果,所以早期识别巨噬细胞活化综合征并进行诊治就显得十分必要。本文通过检索Pubmed数据库,对系统性红斑狼疮合并巨噬细胞活化综合征的诊治进展做一综述。Macrophage activation syndrome (MAS), a hemophagocytic lymphohistiocytosis (HLH) secondary to rheumatic immune disease, is a rare, life-threatening hyperinflammatory disease. Typical clinical symptoms of macrophage activation syndrome include persistent high fever, rash, and enlargement of the liver and spleen, and laboratory tests show a decrease in the number of blood cells in the patient, with high triglycerides or low fibrinogen, ferritinemia, and elevated aminotransferase levels. The incidence of macrophage activation syndrome is high in systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), but more and more scholars have reported the occurrence of macrophage activation syndrome in patients with systemic lupus erythematosus. Macrophage activation syndrome is complex and progresses dangerously, and if it is misdiagnosed or delayed, it may cause irreversible adverse results, so it is necessary to identify and treat macrophage activation syndrome early. This article reviews the progress
目的:总结1例川崎病合并巨噬细胞活化综合征患儿的临床特点及诊疗经过,提高对该疾病的认识。方法:回顾性分析2023年1月山东大学附属威海市立医院儿科收治的1例川崎病合并巨噬细胞活化综合征患儿的临床特点及诊疗经过,并进行相关文献复习。结果:患儿以发热、皮疹及结膜充血为突出表现,在诊断为川崎病后使用静注人免疫球蛋白、阿司匹林治疗,仍高热。加用糖皮质激素后体温降至正常,结合相关检查,诊断KD合并MAS。结论:对于川崎病患儿,若伴有三系减少或静注人免疫球蛋白治疗无效时,应考虑是否合并MAS,及时加用糖皮质激素,降低死亡率。Objective: To summarize the clinical characteristics and treatment outcome of 1 cases of Kawasaki disease complicated by macrophage activation syndrome so as to improve clinician’s awareness of the disease. Methods: Retrospective analysis of the clinical characteristics and diagnosis and treatment process of a child with Kawasaki disease complicated with macrophage activation syndrome admitted to the pediatrics department of Weihai Municipal Hospital affiliated with Shandong University in January 2023, and relevant literature review. Results: The child presented with prominent symptoms of fever, rash, and conjunctival congestion. After diagnosis of Kawasaki disease, intravenous immunoglobulin and aspirin were used for treatment, but the fever persisted. After adding glucocorticoids, the body temperature returned to normal, and combined with relevant examinations, KD was diagnosed with MAS. Conclusions: For children with Kawasaki disease, if there is a reduction in the three lineages or if intravenous immunoglobulin therapy is ineffective, consideration should be given to whether to combine MAS and timely use of glucocorticoids to reduce mortality.
