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Abstract：
Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA), caused by exaggerated but ineffective immune response. The aim of the study was to compare the capacity of the HLH-2004 guidelines with the capacity of the MAS guidelines from 2005, and with the new set of classification criteria from 2016 in diagnosing MAS complicating sJIA. The study included 35 children aged 1-18 diagnosed with sJIA according to ILAR criteria and treated at the Department of Pediatrics, Division of Immunology and Rheu-matology, Zagreb University Hospital Centre, in the period from 2009 to 2015. Out of 35 patients diagnosed with sJIA, there were 12 girls and 23 boys, with the mean age at disease onset (±SD) 5.51±3.65 years. Eight patients had flare of disease. With the guidelines from 2005, MAS was diag-nosed in six (17.1%) patients with sJIA. With the new set of classification criteria from 2016, MAS was diagnosed in four (11.4%) patients with sJIA. MAS was not diagnosed with the HLH-2004 guidelines. In our study, four out of six patients had MAS at the onset of sJIA, and in the rest two it occurred during relapse. Two patients with MAS developed full-blown clinical picture while another four had incomplete clinical features with minor laboratory alteration. Due to the use of different di-agnostic guidelines, we found difference in the prevalence of MAS. It was slightly higher in comparison to available studies, while other researched features, such as clinical characteristics, were similar.
摘要：
巨噬细胞激活综合征（MAS）是系统性幼年特发性关节炎（sJIA）的潜在致命并发症，由夸大但无效的免疫反应引起的。该研究的目的是将HLH-2004指南的能力与2005年MAS指南的能力以及2016年新的分类标准进行比较，以诊断MAS并发sJIA。该研究包括35名根据ILAR标准诊断为sJIA的1-18岁儿童，并在儿科接受治疗。免疫学和Rheu-matology，萨格勒布大学医院中心，2009年至2015年。在诊断为sJIA的35名患者中，有12个女孩和23个男孩，发病时的平均年龄（±SD）为5.51±3.65岁。八名患者出现疾病发作。根据2005年的指南，MAS在6例（17.1％）sJIA患者中进行了诊断。根据2016年的新分类标准，MAS在4例（11.4％）sJIA患者中被诊断出。MAS未被HLH-2004指南诊断。在我们的研究中,六名患者中有四名在sJIA发作时患有MAS，在其余两个中，它发生在复发期间。两名MAS患者的临床表现全面，另外四名患者的临床特征不完整，实验室检查略有改变。由于使用了不同的独立指南，我们发现MAS的患病率存在差异。与现有研究相比，这一数字略高，而其他研究的功能，如临床特征，是相似的。
