Hereditäre periodische Fiebersyndrome (HPFS)

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.