Periodiske febersyndromer

Jannik Helweg-Larsen; Ann-Brit Eg Hansen; Sussi Bagge Mortensen; Isik Somuncu Johansen

Periodiske febersyndromer

Jannik Helweg-Larsen, Ann-Brit Eg Hansen, Sussi Bagge Mortensen, Isik Somuncu Johansen

Institut for Klinisk Medicin

Abstract

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.

Bidragets oversatte titel	Periodic fever syndromes
Originalsprog	Dansk
Artikelnummer	V11170820
Tidsskrift	Ugeskrift for Laeger
Vol/bind	180
Udgave nummer	22
Antal sider	6
ISSN	0041-5782
Status	Udgivet - 2018

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http://ugeskriftet.dk/videnskab/periodiske-febersyndromer

Citationsformater

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T1 - Periodiske febersyndromer

AU - Helweg-Larsen, Jannik

AU - Hansen, Ann-Brit Eg

AU - Mortensen, Sussi Bagge

AU - Johansen, Isik Somuncu

PY - 2018

Y1 - 2018

N2 - 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.

AB - 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.

M3 - Tidsskriftartikel

C2 - 29808817

SN - 0041-5782

VL - 180

JO - Ugeskrift for Laeger

JF - Ugeskrift for Laeger

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Periodiske febersyndromer

Abstract

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