Department of Rheumatology and Immunology, Peking University People's Hospital and Key Laboratory of Rheumatism Mechanism and Immune Diagnosis (BZ0135) and Peking-Tsinghua Center for Life Science, Beijing, China.
Department of Rheumatology, Peking Union Medical College Hospital and Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Clin Rheumatol. 2018 Jan;37(1):93-100. doi: 10.1007/s10067-017-3625-6. Epub 2017 Apr 13.
The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly. Hyperferritinemia, hypoalbuminemia, and hyper-lactate dehydrogenase (LDH)-nemia were among the most common laboratory abnormalities. Compared with pre-MAS visit, patients at the onset of MAS had greater frequencies of renal involvement, liver dysfunction, and cytopenia. Receiver operating characteristic (ROC) analysis identified optimal cutoff values of ferritin (>662.5 ng/mL) and LDH (>359 U/mL) to predict the occurrence of MAS in SLE. SLE flare and infection were the common triggers of MAS in SLE. Abortion and parturition were recorded as well. The overall mortality rate was 12.5%. All patients received corticosteroids. Cyclosporine A, cyclophosphamide, and etoposide were the three most commonly used immunosuppressants. Rituximab was given to one patient. Intravenous immunoglobulin (IVIG) was added for 46.9% patients. MAS is a potentially fatal complication of SLE. Its occurrence is most frequently associated with active SLE disease or infection. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS. Corticosteroids with immunosuppressants and IVIG may be an appropriate treatment.
本研究旨在描述系统性红斑狼疮（SLE）合并巨噬细胞活化综合征（MAS）的临床和实验室特征、诱发因素、治疗方法和转归。采用多中心病例对照研究，对 1997 年至 2014 年期间 6 家三级医院的 32 例 SLE 相关 MAS 患者进行了研究。选择同期无 MAS 发作的 64 例年龄和性别匹配的 SLE 患者作为对照。最常见的临床特征是发热，其次是脾肿大。常见的实验室异常包括血清铁蛋白升高、低白蛋白血症和乳酸脱氢酶（LDH）升高。与 MAS 前就诊相比，MAS 发病时患者更常出现肾脏受累、肝功能异常和血细胞减少。受试者工作特征（ROC）分析确定了铁蛋白（>662.5ng/ml）和 LDH（>359U/ml）的最佳截断值，以预测 SLE 中 MAS 的发生。SLE 活动和感染是 SLE 中 MAS 的常见诱因。还记录了流产和分娩。总的死亡率为 12.5%。所有患者均接受皮质类固醇治疗。环孢素 A、环磷酰胺和依托泊苷是最常用的三种免疫抑制剂。1 例患者接受利妥昔单抗治疗。46.9%的患者加用静脉注射免疫球蛋白（IVIG）。MAS 是 SLE 的一种潜在致命并发症。其发生最常与活动期 SLE 疾病或感染有关。在 SLE 患者中，出现不明原因发热、血细胞减少或肝功能异常，同时铁蛋白和 LDH 水平升高，应怀疑 MAS。皮质类固醇联合免疫抑制剂和 IVIG 可能是一种合适的治疗方法。
