2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative
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DOI：
10.1002/art.39332
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发表时间：
2016-03-01
影响因子：
13.3
通讯作者：
Cron, Randy Q.
中科院分区：
文献类型：
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作者：
Ravelli, Angelo;Minoia, Francesca;Cron, Randy Q.
目的建立系统性幼年特发性关节炎（JIA）患者巨噬细胞活化综合征（MAS）的分型标准。首先，一个由28名专家组成的小组被要求根据疾病发作时的临床和实验室特征将428名患者分类为患有或不患有MAS。428例患者包括161例全身性JIA相关MAS患者和267例可能与MAS混淆的患者（无MAS或全身感染证据的活动性全身性JIA）。接下来，通过评价使用标准产生的分类与专家共识分类之间的一致性，评估候选标准将个体患者分类为患有MAS或未患有MAS的能力。最终的标准在一个共识conference.Results专家达成共识的428例患者配置文件的391（91.4%）的分类。共对982个候选标准进行了统计学检验。在共识会议上评价了从文献中获得的37个最佳性能标准和8个标准。在会议期间，82%的专家就最终的MAS分类标准达成了共识。在验证分析中，这些标准的灵敏度为0.73，特异性为0.99。结论建立了一套MAS合并系统性JIA的分型标准，并初步验证了其有效性。这些标准的使用可能会提高对系统性JIA中MAS的理解，并通过确保适当的患者入组研究来加强发现有效疗法的努力。
Objective To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA).Methods A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference.Results Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (kappa=0.76).Conclusion We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies.