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The TNF Receptor-Associated Autoinflammatory Syndrome (TRAPS)

  • Marco GattornoEmail author
Chapter

Abstract

The TNF receptor-associated autoinflammatory syndrome (TRAPS, MIM 142680), formerly known as familial Hibernian fever [1], is a rare dominantly inherited disorder, caused by mutations in the p55 TNF receptor (or TNFR1), encoded by the TNF superfamily receptor 1A (TNFRSF1A) gene [2]. The disease was originally identified in families of Northern European ancestry but has been described in almost all ethnic groups, including those living in Mediterranean countries and Asia. Fever is often prolonged and can be accompanied by serositis, arthritis, a skin rash with underlying fasciitis and periorbital oedema.

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.UO Pediatria IIGenoaItaly

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