Performance of Tel-Hashomer, Livneh, pediatric and new Eurofever/PRINTO classification criteria for familial Mediterranean fever in a referral center

  • Ayşe Tanatar
  • Hafize Emine Sönmez
  • Şerife Gül Karadağ
  • Figen Çakmak
  • Mustafa Çakan
  • Ferhat Demir
  • Betül Sözeri
  • Nuray Aktay AyazEmail author
Classification Criteria


Until now, the diagnosis of familial Mediterranean fever (FMF) was based on validated subsets of clinical criteria, but recently new Eurofever/PRINTO classification criteria concerning genetic analyses were proposed. The study aimed to compare the performances of three validated diagnostic criteria (Tel-Hashomer, Livneh, pediatric criteria) and new Eurofever/PRINTO classification criteria. The medical charts of study and control groups were reviewed retrospectively. Patients were evaluated for three diagnostic criteria and new Eurofever/PRINTO classification criteria. Control group consists of patients with other autoinflammatory diseases. A total of 1291 patients were classified into three groups according to their mutations: group 1: 447 patients with homozygous mutations; group 2: 429 patients with compound heterozygous mutations; and group 3: 415 patients with one heterozygous mutation. Similar diagnostic utility was found according to Livneh criteria between groups. But, proportion of patients fulfilling Tel-Hashomer and pediatric criteria was higher in groups 1 and 2. According to Eurofever/PRINTO criteria, 98.2% of patients with homozygous mutations, 94.2% of patients with compound heterozygous mutations and 80.2% of patients with heterozygous mutations were classified as FMF. In control group, 99.2% of them fulfilled the Livneh criteria, 66.9% met the pediatric criteria and 0.8% satisfied the Tel-Hashomer criteria, while none of control patients met the Eurofever/PRINTO classification criteria. Performances of three validated diagnostic criteria and new Eurofever/PRINTO classification criteria for FMF were similar and provide high utility in diagnosing/classifying patients with homozygous and compound heterozygous mutations. However, both Eurofever/PRINTO classification criteria and Tel-Hashomer criteria had significantly lower performance in heterozygous patients.


Familial Mediterranean fever Autoinflammation MEFV Diagnostic criteria Classification criteria 


Author contributions

AT conceptualized and designed the study, drafted the initial manuscript, and had full access to all the data in the study; HES designed the study, conducted the data analyses, drafted the initial manuscript, and had full access to all the data in the study; ŞGK, FÇ and MÇ drafted the initial manuscript; NAA conceptualized and designed the study, drafted the final manuscript, and had full access to all the data in the study and all authors reviewed and revised the manuscript and approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Compliance with ethical standards

Conflict of interest

The authors declare that they do have any conflict of interest.

Ethical approval

The study was reviewed and approved by the ethical review committee of Kanuni Sultan Süleyman Training and Research Hospital (Ethics approval number: KAEK/2018.12.65).

Informed consent

All participants and parents provided written informed consent.

Supplementary material

296_2019_4463_MOESM1_ESM.docx (16 kb)
Supplementary material 1 (DOCX 16 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Pediatric Rheumatology, Kanuni Sultan Süleyman Research and Training HospitalUniversity of Health SciencesIstanbulTurkey
  2. 2.Department of Pediatric Rheumatology, Ümraniye Research and Training HospitalUniversity of Health SciencesIstanbulTurkey

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