Cohen E M, D'Silva K, Kreps D, Son M B, Costenbader K H
1 Division of Immunology, 1862 Boston Children's Hospital , Boston, MA, USA.
2 Department of Rheumatology, 1861 Brigham and Women's Hospital , Boston, MA, USA.
Lupus. 2018 Jun;27(7):1065-1071. doi: 10.1177/0961203318759428. Epub 2018 Feb 16.
Background Macrophage activation syndrome (MAS) is an uncommon but serious complication of systemic lupus erythematosus (SLE). We aimed to identify factors associated with MAS among adult hospitalized SLE patients. Methods Within the Brigham and Women's Hospital (BWH) Lupus Center Registry, we identified adult SLE patients > age 17 who had been hospitalized from 1970 to 2016, with either ferritin > 5000 ng/ml during admission or "macrophage activation syndrome" or "MAS" in discharge summary. We confirmed MAS by physician diagnosis in medical record review. We matched each hospitalized SLE patient with MAS to four SLE patients hospitalized without MAS (by SLE diagnosis date ±1 year). We employed conditional logistic regression models to identify clinical factors associated with MAS among hospitalized SLE patients. Results Among 2094 patients with confirmed SLE, we identified 23 who had a hospitalization with MAS and compared them to 92 hospitalized without MAS. Cases and controls had similar age at SLE diagnosis (29.0 vs. 30.5, p = 0.60), and hospital admission (43.0 vs. 38.3, p = 0.80), proportion female (78% vs. 84%, p = 0.55), and time between SLE diagnosis and hospitalization (1971 vs. 1732 days, p = 0.84). Arthritis (OR 0.04 (95% CI 0.004-0.35)) and hydroxychloroquine use (OR 0.18 (95% CI 0.04-0.72)) on admission were associated with decreased MAS risk. Admission Systemic Lupus Erythematosus Disease Activity Index scores (30 vs. 19, p = 0.002) and lengths of stay (16 days vs. 3 days, p < 0.0001) were much higher among cases. Death during hospitalization was 19% among cases and 3% among controls ( p = 0.03). Conclusions In this case-control study of hospitalized adult SLE patients, arthritis and hydroxychloroquine use at hospital admission were associated with decreased MAS risk. Further studies are needed to validate these factors associated with lowered MAS risk.
巨噬细胞活化综合征（MAS）是系统性红斑狼疮（SLE）一种罕见但严重的并发症。我们旨在确定成年住院SLE患者中与MAS相关的因素。方法：在布莱根妇女医院（BWH）狼疮中心登记处，我们确定了1970年至2016年间住院的年龄大于17岁的成年SLE患者，这些患者在入院时铁蛋白>5000 ng/ml，或出院小结中有“巨噬细胞活化综合征”或“MAS”。我们通过病历审查中医生的诊断来确诊MAS。我们将每例住院的SLE合并MAS患者与4例未合并MAS的住院SLE患者进行匹配（按SLE诊断日期±1年）。我们采用条件逻辑回归模型来确定住院SLE患者中与MAS相关的临床因素。结果：在2094例确诊SLE患者中，我们确定了23例合并MAS住院的患者，并将他们与92例未合并MAS住院的患者进行比较。病例组和对照组在SLE诊断时的年龄（29.0对30.5，p = 0.60）、入院时年龄（43.0对38.3，p = 0.80）、女性比例（78%对84%，p = 0.55）以及SLE诊断与住院之间的时间（1971天对1732天，p = 0.84）相似。入院时关节炎（比值比0.04（95%可信区间0.004 - 0.35））和使用羟氯喹（比值比0.18（95%可信区间0.04 - 0.72））与MAS风险降低相关。病例组的入院系统性红斑狼疮疾病活动指数评分（30对19，p = 0.002）和住院时间（16天对3天，p < 0.0001）要高得多。住院期间病例组的死亡率为19%，对照组为3%（p = 0.03）。结论：在这项针对成年住院SLE患者的病例对照研究中，入院时关节炎和使用羟氯喹与MAS风险降低相关。需要进一步研究来验证这些与降低MAS风险相关的因素。
