A case of childhood systemic lupus erythematosus complicated with macrophage activation syndrome and literature review

VernacularTitle:儿童系统性红斑狼疮合并巨噬细胞活化综合征1例报告并文献复习
Author: Linli WANG ¹ ; Fengjun GUAN
Author Information

1. 徐州医科大学附属医院儿科
Keywords: systemic lupus erythematosus; macrophage activation syndrome; child
From: Journal of Clinical Pediatrics 2018;36(5):344-347
CountryChina
Language:Chinese
Abstract: Objective To study the clinical features and treatments of childhood systemic lupus erythematosus (SLE) complicated with macrophage activation syndrome (MAS). Methods Clinical data of a patient with SLE complicated with MAS were retrospectively analyzed, and relevant literatures were reviewed. Results An eight-year-old girl diagnosed as SLE two years ago was admitted to the hospital with complaint of high fever for over one week. Peripheral blood cytopenia, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia and hepatolienomegaly occurred after admission gradually. Thus the child was diagnosed definitely as MAS. Conclusions SLE complicated with MAS is not rare. The disease progresses very rapidly and can be life-threatening, while early diagnosis is very difficult. The key to improve prognosis is early diagnosis and treatment.