Tumor necrosis factor receptor-associated periodic fever syndrome in a 58-year-old man: caution not to discount TRAPS as a diagnosis in older patients

J Clin Rheumatol. 2011 Sep;17(6):325-8. doi: 10.1097/RHU.0b013e31822e092c.

Abstract

Tumor necrosis factor receptor-associated periodic fever syndrome (TRAPS) is a rare systemic autoinflammatory disorder characterized by recurrent episodes of fever and localized inflammation of different organs. The disease is dominantly inherited, with an onset usually in early childhood. We describe a case of a 58-year-old patient with TRAPS caused by the low-penetrance R92Q mutation in TNFRSF1A gene. The patient responded well to anti-tumor necrosis factor α therapy. Although periodic fever syndromes, including TRAPS, mainly begin in early childhood, it is important to consider periodic fever syndrome also in patient presenting at an age older than the average reported case for TRAPS.

Publication types

  • Case Reports

MeSH terms

  • Age Factors
  • Antirheumatic Agents / therapeutic use
  • Etanercept
  • Fever
  • Hereditary Autoinflammatory Diseases / diagnosis*
  • Hereditary Autoinflammatory Diseases / drug therapy*
  • Hereditary Autoinflammatory Diseases / genetics
  • Humans
  • Immunoglobulin G / therapeutic use*
  • Male
  • Middle Aged
  • Mutation / genetics
  • Receptors, Tumor Necrosis Factor / therapeutic use*
  • Receptors, Tumor Necrosis Factor, Type I / genetics
  • Treatment Outcome
  • Tumor Necrosis Factor-alpha / antagonists & inhibitors

Substances

  • Antirheumatic Agents
  • Immunoglobulin G
  • Receptors, Tumor Necrosis Factor
  • Receptors, Tumor Necrosis Factor, Type I
  • Tumor Necrosis Factor-alpha
  • Etanercept

Supplementary concepts

  • Periodic fever, familial, autosomal dominant