Tumor necrosis factor receptor-associated periodic syndrome

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Disease Overview

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an inherited condition characterized by recurrent episodes of fever. Episodes can begin at any age but most often begin in early childhood. Fevers typically last about 3 weeks but can last from a few days to a few months. The amount of time between episodes may vary from weeks to years. Episodes usually occur spontaneously, but are sometimes brought on by a variety of triggers (such as injury, infection, or stress). Symptoms during fever episodes may include abdominal, muscle or joint pains; skin rashes (usually on the limbs); puffiness around the eyes; and inflammation in various areas of the body. Some people develop amyloidosis.[9159] TRAPS is caused by mutations in the TNFRSF1A gene and is inherited in an autosomal dominant manner.[9159][9160] Treatment may include systemic corticosteroids at the beginning of an episode to reduce its severity and duration.[9160]


Synonyms

  • FPF
  • Familial Hibernian fever
  • FHF
  • TRAPS
  • Hibernian fever, familial
  • TNF receptor-associated periodic syndrome
  • Periodic fever, familial, autosomal dominant
  • TNF receptor-associated periodic fever syndrome

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National Organization for Rare Disorders