系统性红斑狼疮患儿和幼年特发性关节炎患儿中的巨噬细胞活化综合征
Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis.
作者信息
Bennett Tellen D, Fluchel Mark, Hersh Aimee O, Hayward Kristen N, Hersh Adam L, Brogan Thomas V, Srivastava Rajendu, Stone Bryan L, Korgenski E Kent, Mundorff Michael B, Casper T Charles, Bratton Susan L
机构信息
Pediatric Critical Care, University of Utah, Salt Lake City, UT 84158-1289, USA.
出版信息
Arthritis Rheum. 2012 Dec;64(12):4135-42. doi: 10.1002/art.34661.
OBJECTIVE
To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA).
METHODS
We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use.
RESULTS
A total of 121 children at 28 children's hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil, and more children with JIA received interleukin-1 antagonists.
CONCLUSION
Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease.
目的
描述合并巨噬细胞活化综合征（MAS）的住院儿童的人口统计学特征、干预措施及预后情况，这些儿童的MAS并发系统性红斑狼疮（SLE）或幼年特发性关节炎（JIA）。
方法
我们进行了一项回顾性队列研究，使用2006年10月1日至2010年9月30日期间儿科健康信息系统（PHIS）数据库中记录的数据。参与者有国际疾病分类第九版临床修订本中MAS以及SLE或JIA的诊断编码。主要结局是住院死亡率（本次住院）。次要结局包括重症监护病房（ICU）入住、重症监护干预措施及药物使用情况。
结果
28家儿童医院的121名儿童符合纳入标准，其中19名儿童患有SLE，102名儿童患有JIA。本次住院死亡率为7%（121例患者中有8例）。ICU入住率（33%）、机械通气率（26%）及血管活性药物治疗率（26%）均较高。与患有JIA的儿童相比，患有SLE的儿童死亡率相似（分别为6%和11%；确切P = 0.6）。与患有JIA的儿童相比，更多患有SLE的儿童接受了ICU护理（63%对27%；P = 0.002）、接受了机械通气（53%对21%；P = 0.003）且存在心血管功能障碍（47%接受血管活性药物治疗对23%；P = 0.02）。患有SLE和JIA的儿童使用环孢素的比例相似，但更多患有SLE的儿童接受了环磷酰胺和霉酚酸酯治疗，更多患有JIA的儿童接受了白细胞介素-1拮抗剂治疗。
结论
在合并MAS的风湿性疾病儿童中，器官系统功能障碍很常见，与患有JIA的儿童相比，患有基础SLE的儿童需要更多的器官系统支持。目前儿科MAS的治疗因基础风湿性疾病而异。
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