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Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis

机译：系统性红斑狼疮患儿和幼年特发性关节炎患儿巨噬细胞活化综合征

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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.
机译：目的描述巨噬细胞活化综合征（MAS）并发系统性红斑狼疮（SLE）或青少年特发性关节炎（JIA）的住院儿童的患者人口统计学，干预措施和结果。方法我们使用2006年10月1日至2010年9月30日在儿科健康信息系统（PHIS）数据库中记录的数据进行了一项回顾性队列研究。参与者具有国际疾病分类，第9修订版，MAS的临床修改诊断代码和任一SLE或JIA。主要结果是医院死亡率（用于入院）。次要结果包括重症监护病房（ICU）入院，重症监护干预和药物使用。结果28所儿童医院的121名儿童符合入选标准，其中19例SLE和102例JIA。指数入院死亡率为7％（121位患者中的8位）。 ICU入院（33％），机械通气（26％）和输卵管/升压治疗（26％）很常见。与JIA患儿相比，SLE患儿的死亡率相似（分别为6％和11％;确切的P = 0.6）。 SLE患者中，接受ICU护理的患者比JIA患者多（63％对27％; P = 0.002），接受机械通气治疗（53％对21％; P = 0.003），并且患有心血管功能障碍（47％对23％接受同种药物） /升压药物治疗; P = 0.02）。 SLE患儿和JIA患儿接受环孢素的发生率相似，但更多SLE患儿接受环磷酰胺和霉酚酸酯，更多JIA患儿接受白介素-1拮抗剂。结论风湿性疾病并发MAS患儿的器官系统功能障碍很常见，潜在SLE患儿比JIA患儿需要更多的器官系统支持。小儿MAS的当前治疗因潜在的风湿病而异。

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