The subject of this thesis is a group of rare hereditary disorders, collectively called auto-inflammatory syndromes, with a specific focus on the hyper-IgD and periodic fever syndrome. The autoinflammatory syndromes are characterized by lifelong recurrent episodes with fever, usually accompanied by other inflammatory symptoms such as abdominal pain, diarrhoea, rash or arthralgia. The inflammatory episodes are separated by symptom-free intervals. This thesis explores several issues involving diagnosis, pathogenesis and treatment of the hyper-IgD and periodic syndrome, TNF-receptor associated periodic syndrome and familial Mediterranean fever syndrome.

Contains fulltext : 58960.pdf (Publisher’s version ) (Open Access)

Promotor : Meer, J.W.M. van der Co-promotor : Drenth, J.P.H.

KUN Katholieke Universiteit Nijmegen, 04 november 2004

206 p.