标题：
系统性红斑狼疮合并噬血细胞综合征:多中心病例对照研究
讲者：
杨月
单位：
北京大学人民医院
播放：
4209
论文摘要：
AIMS To describe the clinical and laboratory features, precipitating factors, treatment and outcome of MAS complicating SLE.
METHODS A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014.
RESULTS A total of 32 patients with SLE/MAS were enrolled. 64 age- and sex-matched SLE patients without MAS were selected as controls. The most frequent clinical features were fever and splenomegaly. Low NK-cell activity, hypoalbuminemia, hyperferritinemia and hyper-LDH-nemia were the most common laboratory abnormalities. All patients displayed macrophage hemophagocytosis in bone marrow aspirates. Compared with pre-MAS visit, patients at the onset of MAS had greater frequencies of renal involvement, liver dysfunction and cytopenia. ROC analysis identified optimal cut-offs of laboratory tests to predict the occurrence of MAS. SLE flare and infection were common triggers. Abortion and parturition were recorded as well. The overall mortality rate was 12.5%. All patients received corticosteroids. CsA, CYC and etoposide were 3 most commonly used immunosuppressants. IVIG was added for 46.9% patients.
CONCLUSION MAS is a potentially fatal complication of SLE. Its occurrence is most frequently associated with SLE activity or infection. The occurrence of unexplained fever, cytopenia and liver dysfunction, when associated with high levels of ferritin and LDH, should raise the suspicion. Corticosteroids with immunosuppressants and IVIG may be an appropriate treatment.
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