TY - JOUR
T1 - Hereditäre periodische Fiebersyndrome (HPFS)
AU - Moog, P.
AU - Heemann, U.
N1 - Publisher Copyright:
© 2016, Springer-Verlag Berlin Heidelberg.
PY - 2016/11/1
Y1 - 2016/11/1
N2 - Hereditary periodic fever syndromes are caused by inherited disorders of innate immunity. The resulting attacks of the recurrent highly inflammatory diseases are usually manifested in early childhood. Of these rare diseases, the most common are familial Mediterranean fever, tumor necrosis factor (TNF) receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome and hyper-IgD syndrome. Although the diseases share certain characteristics (e.g. fever attacks, urticaria, serositis and arthralgia), they can usually be distinguished by the clinical phenotype and at the molecular genetic level. The pharmaceutical treatment, which aims at suppression of inflammatory activity and prevention of amyloid A amyloidosis, varies between the syndromes.
AB - Hereditary periodic fever syndromes are caused by inherited disorders of innate immunity. The resulting attacks of the recurrent highly inflammatory diseases are usually manifested in early childhood. Of these rare diseases, the most common are familial Mediterranean fever, tumor necrosis factor (TNF) receptor-associated periodic syndrome, cryopyrin-associated periodic syndrome and hyper-IgD syndrome. Although the diseases share certain characteristics (e.g. fever attacks, urticaria, serositis and arthralgia), they can usually be distinguished by the clinical phenotype and at the molecular genetic level. The pharmaceutical treatment, which aims at suppression of inflammatory activity and prevention of amyloid A amyloidosis, varies between the syndromes.
KW - Autoinflammatory syndromes
KW - Cryopyrin-associated periodic syndromes
KW - Familial Mediterranean fever
KW - Hyper-IgD syndrome
KW - TNF receptor-associated periodic syndrome
UR - http://www.scopus.com/inward/record.url?scp=84991794144&partnerID=8YFLogxK
U2 - 10.1007/s11560-016-0101-2
DO - 10.1007/s11560-016-0101-2
M3 - Artikel
AN - SCOPUS:84991794144
SN - 1862-040X
VL - 11
SP - 417
EP - 423
JO - Nephrologe
JF - Nephrologe
IS - 6
ER -