期刊名称：Egyptian Journal of Pediatric Allergy and Immunology
印刷版ISSN：1687-1642
电子版ISSN：2314-8934
出版年度：2016
卷号：14
期号：1
页码：33-34
出版社：Egyptian Society of Pediatric Allergy and Immunology
摘要：To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). 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 hav ing 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 wit hout 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 con sensus classification of the experts. The final criteria were selected in a consensus conference . 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 crite ria 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 ( κ = 0.76 ). We have developed a set of classification criteria for MAS complicating systemic JIA and p rovided 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
