TY - JOUR
T1 - Performance of different diagnostic criteria for familial Mediterranean fever in children with periodic fevers
T2 - Results from a multicenter international registry
AU - Demirkaya, Erkan
AU - Saglam, Celal
AU - Turker, Turker
AU - Koné-Paut, Isabelle
AU - Woo, Patricia
AU - Doglio, Matteo
AU - Amaryan, Gayane
AU - Frenkel, J.
AU - Uziel, Yosef
AU - Insalaco, Antonella
AU - Cantarini, L.
AU - Hofer, M.
AU - Boiu, Sorina
AU - Duzova, Ali
AU - Modesto, Consuelo
AU - Bryant, Annette
AU - Rigante, D.
AU - Papadopoulou-Alataki, Efimia
AU - Guillaume-Czitrom, Severine
AU - Kuemmerle-Deschner, Jasmin
AU - Neven, Benedicte
AU - Lachmann, H.
AU - Martini, Alberto
AU - Ruperto, Nicolino
AU - Gattorno, Marco
AU - Ozen, S.
AU - Eurofever Project, Project
PY - 2016/1/1
Y1 - 2016/1/1
N2 - 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.
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.
KW - Autoinflammatory diseases
KW - Children
KW - Familial mediterranean fever
KW - Livneh criteria
KW - Tel Hashomer criteria
KW - Yalcinkaya-Ozen criteria
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U2 - 10.3899/jrheum.141249
DO - 10.3899/jrheum.141249
M3 - Article
VL - 43
SP - 154
EP - 160
JO - Journal of Rheumatology
JF - Journal of Rheumatology
SN - 0315-162X
IS - 1
ER -