巨噬细胞活化综合征作为系统性红斑狼疮的初始表现

Macrophage activation syndrome as initial presentation of systemic lupus erythematosus.

作者信息

Yeap Say-Tin, Sheen Jiunn-Ming, Kuo Ho-Chang, Hwang Kao-Pin, Yang Kuender D, Yu Hong-Ren

机构信息

Department of Pediatrics, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan.

出版信息

Pediatr Neonatol. 2008 Apr;49(2):39-42. doi: 10.1016/S1875-9572(08)60010-8.

DOI:10.1016/S1875-9572(08)60010-8

PMID:18947015

Abstract

Macrophage activation syndrome (MAS) is known to be a severe and potentially life-threatening complication of rheumatic disorder, especially systemic juvenille rheumatoid arthritis. It is very rare for MAS to be an initial presentation of systemic lupus erythematosus (SLE). Here, we report a 14-year-old girl in whom MAS developed as an initial presentation of SLE. With early diagnosis and administration of cyclosporine A, she had a fair outcome. Further testing showed positive anti-dsDNA about 8 months later.

摘要

巨噬细胞活化综合征（MAS）是已知的风湿性疾病尤其是系统性幼年类风湿性关节炎的一种严重且可能危及生命的并发症。MAS作为系统性红斑狼疮（SLE）的初始表现非常罕见。在此，我们报告一名14岁女孩，其MAS作为SLE的初始表现而出现。通过早期诊断和给予环孢素A，她的预后良好。约8个月后进一步检测显示抗双链DNA阳性。