RT Journal Article
SR Electronic
T1 Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP 154
OP 160
DO 10.3899/jrheum.141249
VO 43
IS 1
A1 Erkan Demirkaya
A1 Celal Saglam
A1 Turker Turker
A1 Isabelle Koné-Paut
A1 Pat Woo
A1 Matteo Doglio
A1 Gayane Amaryan
A1 Joost Frenkel
A1 Yosef Uziel
A1 Antonella Insalaco
A1 Luca Cantarini
A1 Michael Hofer
A1 Sorina Boiu
A1 Ali Duzova
A1 Consuelo Modesto
A1 Annette Bryant
A1 Donato Rigante
A1 Efimia Papadopoulou-Alataki
A1 Severine Guillaume-Czitrom
A1 Jasmine Kuemmerle-Deschner
A1 Bénédicte Neven
A1 Helen Lachmann
A1 Alberto Martini
A1 Nicolino Ruperto
A1 Marco Gattorno
A1 Seza Ozen
A1 for The Paediatric Rheumatology International Trials Organisations (PRINTO) and Eurofever Project
YR 2016
UL http://www.jrheum.org/content/43/1/154.abstract
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.