Validation of the new paediatric criteria for the diagnosis of familial Mediterranean fever: data from a mixed population of 100 children from the French reference centre for auto-inflammatory disorders.
OBJECTIVE
We aimed to validate the new paediatric criteria for diagnosis of FMF in a mixed population of 100 French patients.


METHODS
The study group included 100 FMF children from the French reference centre for auto-inflammatory disorders. A control group of 40 patients with unexplained recurrent fever was reviewed in parallel. Both groups of patients were assessed for both the Tel Hashomer and the new paediatric criteria published by Yalcinkaya et al.


RESULTS
Comparison of Tel Hashomer vs Yalcinkaya's criteria in both groups gave a sensitivity of 99 vs 100%, a specificity of 45 vs 50%, a positive predictive value (PPV) of 81.8 vs 83.3% and a negative predictive value (NPV) of 94.7 vs 100%. However, when we used at least three Yalcinkaya's criteria we obtained a sensitivity of 77% and a specificity of 95% with a PPV of 97.3% and an NPV of 62.3%. The number of mutations in the MEFV gene did not modify results for both sets of criteria.


CONCLUSIONS
The new paediatric Turkish criteria did not make a better contribution to FMF diagnosis than the Tel Hashomer criteria in our mixed population of French children while using an appropriate control group. However, if needed, they can be applied using at least three criteria, which slightly decreases their sensitivity but markedly increases their specificity.
Kondi
Anuela
A
Department of Pediatrics and Pediatric Rheumatology, National Reference Center for Auto-inflammatory Disorders, Bicêtre University Paris Sud Hospital, Le Kremlin-Bicêtre, France.
Hentgen
Véronique
V
Piram
Maryam
M
Letierce
Alexia
A
Guillaume-Czitrom
Séverine
S
Koné-Paut
Isabelle
I
D000293:Adolescent; D000367:Age Factors; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003937:Diagnosis, Differential; D010505:Familial Mediterranean Fever / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D008297:Male; D009154:Mutation; D010641:Phenotype; D011110:Polymorphism, Genetic / Q000235:genetics*; D012680:Sensitivity and Specificity; D013997:Time Factors