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IL-18 serum concentration is continuously elevated in typical familial Mediterranean fever with M694I mutation and can distinguish atypical type

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Pediatric Rheumatology201513 (Suppl 1) :P80

  • Published:


  • Serum Concentration
  • Gene Analysis
  • Kawasaki Disease
  • Familial Mediterranean Fever
  • Cytokine Profile


Familial Mediterranean fever (FMF) can be classified into typical and incomplete/atypical types based on clinical findings and gene analysis, although biomarkers that distinguish typical from atypical FMF have not been unclear.


We here investigated the serum cytokine profiles of IL-1β, IL-6, IL-8, TNF-α, IFN-γ, and IL-18 in FMF compared with those in Kawasaki disease.


IL-1β, IL-6, IL-8, TNF-α, and IFN-γ were not increased in either type of FMF in the remission state and in controls, and IL-6 was elevated during attack periods among patients. Serum IL-18 levels were significantly higher in typical FMF patients with M694I MEFV mutation in remission than in controls at the same level as flared Kawasaki disease, which further increased during attack periods. In contrast, IL-18 levels in atypical FMF with P369S-R408Q mutation or in typical FMF without M694I mutation was not increased, in either disease states.


Thus, serum IL-18 levels at attack increase more than in remission, and that are an excellent marker to distinguish between the two types of FMF.

Authors’ Affiliations

Saitama Medical Center, Saitama Medical University, Pediatrics, Kawagoe, Japan
Shinshu University Graduate School of Medicine, Infection and Host Defense, Matsumoto, Japan
Shinshu University School of Medicine, Pediatrics, Matsumoto, Japan
Northern Yokohama Hospital, Showa University, Children's Medical Center, Yokohama, Japan
Shinshu University School of Medicine, Internal Medicine, Matsumoto, Japan
Ehime University Graduate School of Medicine and Ehime Proteo-medicine Research Center, Pathology, Toon, Japan
Nagasaki Medical Center, Clinical Research Center, Omura, Japan


© Yamazaki et al. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated.