Shi Nana, Wang Xiangying, Zou Lixia, Yang Xinghui, Ma Qian, Lu Meiping
Department of Hematology-Oncology, Children' Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China.
Hangzhou Children' Hospital, Hangzhou, China.
Front Pediatr. 2021 Dec 14;9:767115. doi: 10.3389/fped.2021.767115. eCollection 2021.
Macrophage activation syndrome (MAS) and widespread brain lesions are rare and severe complications of childhood-onset systemic lupus erythematosus (SLE). We report an 11-year-old girl who presented with recurrent rashes for half a year and fever for 2 weeks. Clinical and laboratory features at admission pointed to the diagnosis of SLE and SLE-associated MAS. Cerebral magnetic resonance imaging taken on day 4 after admission showed abnormal signals. Glucocorticoid therapy was started on day 5. Two days later, the patient appeared weak and ill, then the next day she exhibited dizziness, drowsiness, apathia, and dysarthria. High-dose methylprednisolone, cyclophosphamide, and intravenous immunoglobulin were used to treat the patient, and intrathecal dexamethasone was given. The patient was discharged on day 30 after admission and showed complete clinical resolution and improved magnetic resonance imaging resolution.
巨噬细胞活化综合征（MAS）和广泛性脑损伤是儿童期系统性红斑狼疮（SLE）罕见且严重的并发症。我们报告一名11岁女孩，她出现反复皮疹半年，发热2周。入院时的临床和实验室检查结果提示诊断为SLE及SLE相关的MAS。入院后第4天行脑部磁共振成像显示信号异常。第5天开始使用糖皮质激素治疗。两天后，患者出现虚弱和不适，次日出现头晕、嗜睡、淡漠和构音障碍。使用大剂量甲泼尼龙、环磷酰胺和静脉注射免疫球蛋白治疗患者，并给予鞘内注射地塞米松。患者入院后第30天出院，临床症状完全缓解，磁共振成像分辨率提高。
