PT - JOURNAL ARTICLE AU - Erkan Demirkaya AU - Celal Saglam AU - Turker Turker AU - Isabelle Koné-Paut AU - Pat Woo AU - Matteo Doglio AU - Gayane Amaryan AU - Joost Frenkel AU - Yosef Uziel AU - Antonella Insalaco AU - Luca Cantarini AU - Michael Hofer AU - Sorina Boiu AU - Ali Duzova AU - Consuelo Modesto AU - Annette Bryant AU - Donato Rigante AU - Efimia Papadopoulou-Alataki AU - Severine Guillaume-Czitrom AU - Jasmine Kuemmerle-Deschner AU - Bénédicte Neven AU - Helen Lachmann AU - Alberto Martini AU - Nicolino Ruperto AU - Marco Gattorno AU - Seza Ozen TI - Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry AID - 10.3899/jrheum.141249 DP - 2015 Nov 15 TA - The Journal of Rheumatology PG - jrheum.141249 4099 - http://www.jrheum.org/content/early/2015/11/09/jrheum.141249.short 4100 - http://www.jrheum.org/content/early/2015/11/09/jrheum.141249.full AB - Objective Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF). Methods Pediatric patients with FMF from the Eurofever registry were used for the validation of the existing criteria. The other periodic fevers served as controls: mevalonate kinase deficiency (MKD), tumor necrosis factor receptor–associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA), and undefined periodic fever from the same registry. The performances of Tel Hashomer, Livneh, and the Yalcinkaya-Ozen criteria were assessed. Results The FMF group included 339 patients. The control group consisted of 377 patients (53 TRAPS, 45 MKD, 32 CAPS, 160 PFAPA, 87 undefined periodic fevers). Patients with FMF were correctly diagnosed using the Yalcinkaya-Ozen criteria with a sensitivity rate of 87.4% and a specificity rate of 40.7%. On the other hand, Tel Hashomer and Livneh criteria displayed a sensitivity of 45.0 and 77.3%, respectively. Both of the latter criteria displayed a better specificity than the Yalcinkaya-Ozen criteria: 97.2 and 41.1% for the Tel Hashomer and Livneh criteria, respectively. The overall accuracy for the Yalcinkaya-Ozen criteria was 65 and 69.6% (using 2 and 3 criteria), respectively. Ethnicity and residence had no effect on the performance of the Yalcinkaya-Ozen criteria. Conclusion The Yalcinkaya-Ozen criteria yielded a better sensitivity than the other criteria in this international cohort of patients and thus can be used as a tool for FMF diagnosis in pediatric patients from either the European or eastern Mediterranean region. However, the specificity was lower than the previously suggested adult criteria.