Aoyama-Maeda Natsuki, Horino Taro, Ichii Osamu, Terada Yoshio
Department of Endocrinology, Metabolism and Nephrology, Kochi Medical School, Kochi, University, Kohasu, Oko-cho, Nankoku,Kochi 783-8505, Japan.
Laboratory of Anatomy, Department of Biomedical Sciences, Graduate School of Veterinary Medicine, Hokkaido University, Kita 18, Nishi 9, Kita-Ku,Sapporo, 060-0818, Japan.
Rom J Intern Med. 2018 Jun 1;56(2):117-121. doi: 10.1515/rjim-2017-0043.
Macrophage activation syndrome (MAS), a variant of secondary hemophagocyticlymphohistiocytosis, is a potentially life-threatening complication of inflammatory and autoimmune diseases. We present a case of MAS as a rare manifestation of systemic lupus erythematosus. Although initial treatment with corticosteroid, with or without cyclosporine A, is justified in patients with MAS, evidence regarding the effectiveness of this treatment protocol remains to be clarified. Our patient was successfully treated with a combination of intravenous immunoglobulin therapy and intravenous methyl predonisolone pulse therapy, which was followed by a course of oral prednisolone and oral tacrolimus. Based on our experience, we propose tacrolimus to provide a more useful adjuvant treatment to corticosteroid therapy than cyclosporine A.
巨噬细胞活化综合征（MAS）是继发性噬血细胞性淋巴组织细胞增生症的一种变体，是炎症性和自身免疫性疾病的一种潜在危及生命的并发症。我们报告一例MAS作为系统性红斑狼疮的罕见表现。尽管对于MAS患者，使用皮质类固醇（无论是否联合环孢素A）进行初始治疗是合理的，但该治疗方案的有效性证据仍有待阐明。我们的患者通过静脉注射免疫球蛋白治疗和静脉注射甲泼尼龙冲击治疗联合成功治愈，随后接受了一个疗程的口服泼尼松龙和口服他克莫司治疗。基于我们的经验，我们提出与环孢素A相比，他克莫司可为皮质类固醇治疗提供更有效的辅助治疗。
