TY - CHAP
T1 - Clinical Picture in Adulthood and Unusual and Peculiar Clinical Features of FMF
AU - Ben-Zvi, Ilan
AU - Lidar, Merav
AU - Giat, Eitan
AU - Kukuy, Olga
AU - Zafrir, Yaron
AU - Grossman, Chagai
AU - Rosman, Yossi
AU - Livneh, Avi
N1 - Publisher Copyright:
© 2015, Springer International Publishing Switzerland.
PY - 2015
Y1 - 2015
N2 - 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.
AB - 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.
KW - Expand Disability Status Scale
KW - Familial Mediterranean Fever
KW - Familial Mediterranean Fever Patient
KW - Multiple Sclerosis Patient
KW - Posterior Reversible Encephalopathy Syndrome
UR - http://www.scopus.com/inward/record.url?scp=85028574884&partnerID=8YFLogxK
U2 - 10.1007/978-3-319-14615-7_4
DO - 10.1007/978-3-319-14615-7_4
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AN - SCOPUS:85028574884
T3 - Rare Diseases of the Immune System
SP - 47
EP - 80
BT - Rare Diseases of the Immune System
PB - Springer Nature
ER -