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Rheumatology Advance Access originally published online on February 4, 2009
Rheumatology 2009 48(4):395-398; doi:10.1093/rheumatology/ken509
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© The Author 2009. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

A new set of criteria for the diagnosis of familial Mediterranean fever in childhood

Fatos Yalçinkaya1,*, Seza Özen2,*, Zeynep Birsin Özçakar1, Nuray Aktay2, Nilgün Çakar3, Ali Düzova2, Özgür Kasapçopur4, Atilla H. Elhan5, Beyza Doganay5, Mesiha Ekim1, Nazli Kara3, Nermin Uncu3 and Aysin Bakkaloglu2

1Department of Pediatric Nephrology, Ankara University School of Medicine, 2Department of Pediatric Nephrology–Rheumatology, Hacettepe University School of Medicine, 3Department of Pediatric Nephrology, Ministry of Health Diskapi Children's Hospital, Ankara, 4Department of Pediatric Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul and 5Department of Biostatistics, Ankara University School of Medicine, Ankara, Turkey.

Correspondence to: Fatos Yalçinkaya, Çinar Sitesi 5.Blok No:62, Ümitköy 06530, Ankara, Turkey. E-mail: yalcinkaya{at}tr.net


   Abstract

Objectives. Several sets of criteria mainly for adults have been proposed for the diagnosis of FMF. The aim of the present study is to validate the most widely used diagnostic ‘Tel Hashomer’ criteria in children and to establish a new set of criteria for use in childhood.

Methods. The study group consisted of 170 recently diagnosed FMF patients who had mutations at both alleles. They were interviewed about the presence of 35 features and manifestations of FMF at the time of diagnosis. Controls were consecutive patients without FMF (n = 141) who had episodes of fever and clinical features mimicking that of FMF. The diagnostic performance of the candidate features was assessed by multiple logistic regression analysis.

Results. The sensitivity and specificity of Tel Hashomer criteria in our study group were 98.8 and 54.6%, respectively. The multiple logistic regression analysis showed that 5 (fever, abdominal pain, chest pain, arthritis and family history of FMF) of the 35 candidate criteria discriminate FMF from controls with a sensitivity and specificity of 88.8 and 92.2%, respectively. The presence of two or more of these five criteria diagnosed FMF with a sensitivity of 86.5% and a specificity of 93.6%.

Conclusion. It was demonstrated that although the Tel Hashomer criteria were successful in diagnosing the FMF patients in childhood, its specificity was definitely low in children. The new set of criteria has a high sensitivity and specificity for the diagnosis of FMF and is practical to use on an everyday basis.

KEY WORDS: Paediatric, FMF, Diagnostic criteria, Tel Hashomer criteria


*Fatos Yalçinkaya and Seza Özen contributed equally to this study.

Submitted 24 April 2008; revised version accepted 18 December 2008.
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