Familial Mediterranean fever (FMF) is classically manifested with painful, irregularly recurrent, short-lasting bouts of serosal inflammation (mainly peritonitis, synovitis or pleuritis), accompanied by fever, and resolving spontaneously [1]. A rise then fall of acute-phase reactants is typical, but some patients might suffer from continuous inflammation and its associated manifestations [2]. The disease onset is usually in childhood or adolescence, but, in some patients, the initial symptoms develop later in life, during the ages of 20–40 years and sometimes even later [3]. In general, late-onset FMF has a mild phenotype and its genotype lacks the homozygous M694V genotype [3]. The clinical manifestations may vary between patients, including members of the same family and even between identical twins [4, 5]. Clinical diversity may also be seen in the same individual, with different site of attacks in different episodes.
CITATION STYLE
Ben-Zvi, I., Lidar, M., Giat, E., Kukuy, O., Zafrir, Y., Grossman, C., … Livneh, A. (2015). Clinical Picture in Adulthood and Unusual and Peculiar Clinical Features of FMF. In Rare Diseases of the Immune System (Vol. 3, pp. 47–80). Springer Nature. https://doi.org/10.1007/978-3-319-14615-7_4
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