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Monday 27 March 2017

TNF receptor-associated periodic syndrome, familial hibernian fever, TNF Receptor Associated Periodic Fever

TNF Receptor Associated Periodic Fever (TRAPS), formerly known as Familial Hibernian fever, results from a defective gene on chromosome 12p that encodes the cellular membrane receptor protein for TNF (TNFRSF1A).

Most of the 46 known mutations are at the extracellular portion of the protein.

One proposed mechanism for TRAPS-associated inflammation is sustained TNF stimulation of target cells due to impaired shedding of the p55 component of this receptor protein.

A rare disorder, TRAPS was originally described as occurring among individuals of Irish and Scottish descent but patients of other ancestries have since been reported.

Febrile episodes last anywhere from 1 to 6 weeks and as in FMF, sterile peritonitis, pleuritis and arthritis form part of the clinical constellation.

Quite distinctive of TRAPS is the concurrence of severe myalgias and migratory lymphedema during attacks as a consequence of a monocytic fasciitis.

Other unusual features of this disorder are conjunctivitis and the development of inguinal hernias in males.

TRAPS patients also may evidence erythematous rashes during attacks.

A small minority of patients develop amyloidosis.

There are no diagnostic laboratory tests for TRAPS.


- germline mutations in TNFRSF1A gene encoding tumor necrosis factor receptor-1


Anti-inflammatory drugs, NSAIDs and glucocordicoids are used to terminate attacks. Etanercept is recommended as a form of chronic therapy to prevent attacks. Anakinra has also been reported to suppress ongoing attacks.

See also

- hereditary autoinflammatory diseases


- A novel Y331X nonsense mutation in TNFRSF1A gene in two unrelated Turkish families with periodic fever syndrome. Kutukculer N, Gulez N, Karaca N, Aksu G, Berdeli A. Int J Immunogenet. 2009 Oct 5. PMID: 19804406

- TNF receptor-associated periodic syndrome (TRAPS): A new cause of joint destruction? Schoindre Y, Feydy A, Giraudet-Lequintrec JS, Kahan A, Allanore Y. Joint Bone Spine. 2009 Sep 29. PMID: 19796978

- Multiple sclerosis. TNFRSF1A, TRAPS and multiple sclerosis. Kümpfel T, Hohlfeld R. Nat Rev Neurol. 2009 Oct;5(10):528-9. PMID: 19794511