严重且危及生命的全身性红斑狼疮发作。
Severe and life-threatening onset of systemic lupus erythematosus.
机构信息
Department of Rheumatology, Centro Hospitalar Universitário de São João, Porto, Portugal; Department of Medicine, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
Department of Rheumatology, Centro Hospitalar Universitário de São João, Porto, Portugal; Department of Medicine, Faculdade de Medicina da Universidade do Porto, Porto, Portugal.
出版信息
Reumatol Clin (Engl Ed). 2023 Aug-Sep;19(7):402-403. doi: 10.1016/j.reumae.2022.09.003.
Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases. We report a unique case of a previously healthy 20-year-old female presenting with MAS as first presentation of systemic lupus erythematosus. Remission was achieved with hydroxychloroquine, intravenous methylprednisolone pulse followed by oral prednisolone and cyclosporine. However, the management of MAS is still challenging, and the mortality rate remains high.
巨噬细胞活化综合征（MAS）是风湿性疾病潜在的危及生命的并发症。我们报告了一例罕见的病例，一名此前健康的 20 岁女性以 MAS 为首发表现，出现全身性红斑狼疮。经羟氯喹、静脉注射甲基强的松龙脉冲治疗后，继以口服泼尼松龙和环孢素治疗，病情缓解。然而，MAS 的治疗仍然具有挑战性，死亡率仍然很高。
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