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01-07-2015 | Review

The myths we believed in familial Mediterranean fever: what have we learned in the past years?

Authors: Seza Ozen, Ezgi Deniz Batu

Published in: Seminars in Immunopathology | Issue 4/2015

Abstract

Familial Mediterranean fever is the most common monogenic periodic fever syndrome over the world especially in the eastern Mediterranean. It presents with recurrent and self-limited inflammatory attacks of fever and polyserositis along with high acute-phase reactants. The disease is associated with mutations in the MEFV gene that encodes pyrin, a component of inflammasome, which leads to exaggerated inflammatory response through uncontrolled production of interleukin 1. With the identification of the gene associated with the disease, we believed that everything was solved and that this was an ordinary monogenic disease with autosomal recessive inheritance. However, through the breathtaking progress in the basic research field as well as the clinical care of these patients, we have understood that the picture for this monogenic disorder was more complicated than we had anticipated. In this review, we have discussed the myths we believed in familial Mediterranean fever and how they have evolved during the past years.

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Title: The myths we believed in familial Mediterranean fever: what have we learned in the past years?
Authors: Seza Ozen
Ezgi Deniz Batu
Publication date: 01-07-2015
Publisher: Springer Berlin Heidelberg
Published in: Seminars in Immunopathology / Issue 4/2015
Print ISSN: 1863-2297
Electronic ISSN: 1863-2300
DOI: https://doi.org/10.1007/s00281-015-0484-6

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