Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders
Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders
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DOI：
10.1136/adc.85.5.421
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发表时间：
2001-11-01
影响因子：
5.2
通讯作者：
Murray, KJ
中科院分区：
文献类型：
--
作者：
Sawhney, S;Woo, P;Murray, KJ
目的：回顾巨噬细胞激活综合征（MAS）的诱发事件、临床特征、治疗和预后。方法：回顾性分析1980 - 2000年前瞻性收集的风湿病儿童数据库中MAS病例。结果：9例患者（8例女孩）被认为有MAS的证据。初步诊断为全身性发作的青少年特发性关节炎7例，鼻炎相关关节炎1例，慢性婴儿神经皮肤关节综合征1例。平均发病年龄为5.7岁，MAS前持续时间为4.2年。没有药物被确定为诱因。8人在MAS之前感染；其中4例确定了特定的感染原。高热、新发肝脾肿大和淋巴结病变是常见的临床特征。血小板计数急剧下降，从平均346降到99 × 10(9)/升。平均红细胞沉降率（3例）从115 mm/h降至28 mm/h。病程中肝功能异常8例，凝血功能障碍6例。7例患者中有4例骨髓检查支持明确的噬血细胞症诊断。所有患者均接受大剂量类固醇治疗（8例静脉注射，1例口服），5例环孢素，2例环磷酰胺和1例抗胸腺细胞球蛋白。三名严重肾功能受损的患者中有两名死亡。结论：mas是一种罕见且可能致命的儿童风湿病并发症。患者多为女性，且多为感染前病例。骨髓研究支持这一诊断。肾功能紊乱可能是预后不良的征兆。积极的早期治疗是必不可少的。
Aims-To review the precipitating events, clinical features, treatment, and outcome of macrophage activation syndrome (MAS).Methods-Retrospective review of cases of MAS from a prospectively collected database of children with rheumatic diseases from 1980 to 2000.Results-Nine patients (eight girls) were considered to have evidence of MAS. The primary diagnosis was systemic onset juvenile idiopathic arthritis in seven, enthesitis related arthritis in one, and chronic infantile neurological cutaneous articular syndrome in one. Mean age of onset was 5.7 years, and duration prior to MAS, 4.2 years. No medication was identified as a trigger. Eight had infections prior to MAS; specific infectious agents were identified in four. High grade fever, new onset hepatosplenomegaly, and lymphadenopathy were common clinical features. Platelet counts fell dramatically, from an average of 346 to 99 x 10(9)/l. Mean erythrocyte sedimentation rate (in three patients) fell from 115 to 28 mm/h. Eight had abnormal liver function during the disease course, and six had coagulopathy. Bone marrow examination supported the diagnosis with definite haemophagocytosis in four of seven. All received high dose steroids (eight intravenous, one oral), five cyclosporin, two cyclophosphamide, and one antithymocyte globulin. Two of three patients with significant renal impairment died.Conclusion-MAS is a rare and potentially fatal complication of childhood rheumatic disorders. Most of our patients were female, and most cases were preceded by infection. Bone marrow studies support the diagnosis. Deranged renal function may be a poor prognostic sign. Aggressive early therapy is essential.