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Periodiske febersyndromer

Jannik Helweg-Larsen1, Ann-Brit Eg Hansen2, Sussi Bagge Mortensen3, 4 & Isik Somuncu Johansen4 1) Infektionsmedicinsk Afdeling, Rigshospitalet 2) Infektionsmedicinsk Afdeling, Hvidovre Hospital 3) Klinisk Immunologisk Afdeling, Odense Universitetshospital 4) Infektionsmedicinsk Afdeling, Odense Universitetshospital Ugeskr Læger 2018;180:V11170820
Ugeskr Læger 2018;180:V11170820
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Periodic fever syndromes
Periodic fever syndromes (PFS) are characterised by recurrent fever and excessive systemic inflammation. These rare and hereditary syndromes include familial Mediterranean fever, tumour necrosis factor receptor-1 associated periodic syndrome, mevalonate kinase deficiency and cryopyrin-associated periodic syndrome. Each PFS has distinct clinical and genetic features. Availability of improved genetic methods has improved the understanding of the syndromes and diagnostic testing. The main complication is systemic amyloidosis. Targeted biological therapy such as interleukin-1 inhibitors is emerging as an effective treatment.
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