TY - JOUR T1 - Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 799 LP - 805 DO - 10.1136/annrheumdis-2014-206580 VL - 74 IS - 5 AU - Silvia Federici AU - Maria Pia Sormani AU - Seza Ozen AU - Helen J Lachmann AU - Gayane Amaryan AU - Patricia Woo AU - Isabelle Koné-Paut AU - Natacha Dewarrat AU - Luca Cantarini AU - Antonella Insalaco AU - Yosef Uziel AU - Donato Rigante AU - Pierre Quartier AU - Erkan Demirkaya AU - Troels Herlin AU - Antonella Meini AU - Giovanna Fabio AU - Tilmann Kallinich AU - Silvana Martino AU - Aviel Yonatan Butbul AU - Alma Olivieri AU - Jasmin Kuemmerle-Deschner AU - Benedicte Neven AU - Anna Simon AU - Huri Ozdogan AU - Isabelle Touitou AU - Joost Frenkel AU - Michael Hofer AU - Alberto Martini AU - Nicolino Ruperto AU - Marco Gattorno AU - for the Paediatric Rheumatology International Trials Organisation (PRINTO) and Eurofever Project Y1 - 2015/05/01 UR - http://ard.bmj.com/content/74/5/799.abstract N2 - The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS); cryopyrin-associated periodic syndromes (CAPS)) enrolled in the Eurofever Registry up until March 2013 were evaluated. Patients with periodic fever, aphthosis, pharyngitis and adenitis (PFAPA) syndrome were used as negative controls. For each genetic disease, patients were considered to be ‘gold standard’ on the basis of the presence of a confirmatory genetic analysis. Clinical criteria were formulated on the basis of univariate and multivariate analysis in an initial group of patients (training set) and validated in an independent set of patients (validation set). A total of 1215 consecutive patients with periodic fevers were identified, and 518 gold standard patients (291 FMF, 74 MKD, 86 TRAPS, 67 CAPS) and 199 patients with PFAPA as disease controls were evaluated. The univariate and multivariate analyses identified a number of clinical variables that correlated independently with each disease, and four provisional classification scores were created. Cut-off values of the classification scores were chosen using receiver operating characteristic curve analysis as those giving the highest sensitivity and specificity. The classification scores were then tested in an independent set of patients (validation set) with an area under the curve of 0.98 for FMF, 0.95 for TRAPS, 0.96 for MKD, and 0.99 for CAPS. In conclusion, evidence-based provisional clinical criteria with high sensitivity and specificity for the clinical classification of patients with inherited periodic fevers have been developed. ER -