Development and initial validation of the MS score for diagnosis of macrophage activation syndrome in systemic juvenile idiopathic arthritis.

Autor:	Minoia F; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy francesca.minoia@policlinico.mi.it.; Istituto Giannina Gaslini, Genova, Italy., Bovis F; Università degli Studi di Genova, Genova, Italy., Davì S; Istituto Giannina Gaslini, Genova, Italy., Horne A; Karolinska University Hospital Solna, Stockholm, Sweden., Fischbach M; Hopital Universitaire Hautepierre, Strasbourg, France., Frosch M; School of Medicine Witten/Herdecke, Datteln, Germany., Huber A; IWK Health Centre, Halifax, Nova Scotia, Canada., Jelusic M; University of Zagreb School of Medicine, Zagreb, Croatia., Sawhney S; Sir Ganga Ram Hospital, New Delhi, India., McCurdy DK; Mattel Children's Health Center UCLA, Los Angeles, California, USA., Silva CA; Childrens' Institute, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil., Rigante D; Fondazione Policlinico Universitario A Gemelli IRCCS, Roma, Italy.; Università Cattolica del Sacro Cuore, Roma, Italy., Unsal E; Dokuz Eylu ̈l University Medical School, Izmir, Turkey., Ruperto N; Istituto Giannina Gaslini, Genova, Italy., Martini A; Istituto Giannina Gaslini, Genova, Italy.; Università degli Studi di Genova, Genova, Italy., Cron RQ; University of Alabama at Birmingham, Birmingham, Alabama, USA., Ravelli A; Istituto Giannina Gaslini, Genova, Italy.; Università degli Studi di Genova, Genova, Italy.; Sechenov First Moscow State Medical University, Moscow, Russian Federation.
Jazyk:	angličtina
Zdroj:	Annals of the rheumatic diseases [Ann Rheum Dis] 2019 Oct; Vol. 78 (10), pp. 1357-1362. Date of Electronic Publication: 2019 Jul 11.
DOI:	10.1136/annrheumdis-2019-215211
Abstrakt:	Objective: To develop and validate a diagnostic score that aids in identifying macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (sJIA). Methods: The clinical and laboratory features of 362 patients with sJIA-associated MAS and 404 patients with active sJIA without evidence of MAS were collected in a multinational collaborative project. Eighty percent of the study population was used to develop the score and the remaining 20% constituted the validation sample. A Bayesian Model Averaging approach was used to assess the role of each clinical and laboratory variables in the diagnosis of MAS and to obtain the coefficients of selected variables. The final score, named MAS/sJIA (MS) score, resulted from the linear combination of these coefficients multiplied by the values of each variable. The cut-off that best discriminated MAS from active sJIA was calculated by means of receiver operating characteristic (ROC) curve analysis. Score performance was evaluated in both developmental and validation samples. Results: The MS score ranges from -8.4 to 41.8 and comprises seven variables: central nervous system dysfunction, haemorrhagic manifestations, active arthritis, platelet count, fibrinogen, lactate dehydrogenase and ferritin. A cut-off value ≥-2.1 revealed the best performance in discriminating MAS from active sJIA, with a sensitivity of 0.85, a specificity of 0.95 and a kappa value of 0.80. The good performance of the MS score was confirmed in the validation sample. Conclusion: The MS score is a powerful and feasible tool that may assist practitioners in making a timely diagnosis of MAS in patients with sJIA. Competing Interests: Competing interests: None declared. (© Author(s) (or their employer(s)) 2019. No commercial re-use. See rights and permissions. Published by BMJ.)
Databáze:	MEDLINE
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