Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatologic diseases. Data regarding the clinical course, management and outcome of adults with MAS is limited. Therefore, we aimed to describe the clinical features, treatment and outcome of adult patients with MAS, and review the literature for previous cohorts. We retrospectively reviewed patients with MAS complicating rheumatologic diseases between the years 2007 and 2017. Through Pubmed, Medline and Scopus literature search we identified previous cases of adult patients with MAS. We identified 7 patients with MAS complicating rheumatologic diseases (5 females and 2 males). The median age of diagnosis was 32 (range 26-57) years. The median follow-up was 30 months (range 6.95-36.5) months. The underlying rheumatologic disease was adult onset Still's disease (AOSD) in 3 patients, systemic juvenile idiopathic arthritis (sJIA) in 2 patients, systemic lupus erythematosus (SLE) in 1 patient, and systemic vasculitis in 1 patient. Four patients developed MAS concurrently with the clinical development of the rheumatologic disease. All the patients were treated with systemic corticosteroids. Five patients were treated with cyclosporine A, one of which received combination therapy with anakinra, and one received tocilizumab. Two patients deceased during the hospitalization. We identified 92 patients from literature cohorts, 73 (79%) of them with AOSD. MAS developed concurrently with the underlying rheumatologic disease in 25 (27%) patients, and 30 (33%) patients deceased. Our cohort and previous cohorts demostrate that MAS often presents concurrently with the underlying rheumatologic disease and is associated with a high mortality rate. Further larger prospective studies are needed to determine the optimal management of MAS.

中文翻译：

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成人巨噬细胞活化综合征使风湿性疾病复杂化：基于病例的综述。

巨噬细胞活化综合症（MAS）是风湿性疾病威胁生命的并发症。关于成人MAS的临床过程，治疗和结局的数据有限。因此，我们旨在描述成年MAS患者的临床特征，治疗和结局，并回顾以前的队列研究文献。我们回顾性研究了2007年至2017年间患有风湿性疾病的MAS复杂患者。通过Pubmed，Medline和Scopus文献检索，我们确定了成年MAS成人患者。我们确定了7例患有风湿性疾病的MAS患者（5例女性和2例男性）。诊断的中位年龄为32岁（范围26-57）。中位随访时间为30个月（范围6.95-36.5）。潜在的风湿病是成年Still' 病（AOSD）3例，系统性幼年特发性关节炎（sJIA）2例，系统性红斑狼疮（SLE）1例，系统性血管炎1例。四名患者在风湿性疾病的临床发展的同时发生了MAS。所有患者均接受全身性激素治疗。5例患者接受环孢霉素A治疗，其中1例接受anakinra联合治疗，1例接受tocilizumab治疗。两名患者在住院期间死亡。我们从文献队列中鉴定出92例患者，其中73例（79％）患有AOSD。25例（27％）患者和30例（33％）患者死亡，MAS与潜在的风湿病同时发生。我们的队列和先前的队列表明，MAS经常与潜在的风湿病同时出现，并伴有较高的死亡率。需要进一步的前瞻性研究来确定MAS的最佳管理。