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Failure of sustained response to etanercept and refractoriness to anakinra in patients with T50M TNF-receptor-associated periodic syndrome
  1. N Quillinan1,
  2. G Mannion1,
  3. A Mohammad1,
  4. R Coughlan1,
  5. L J Dickie2,
  6. M F McDermott2,
  7. D McGonagle1,2
  1. 1Department of Rheumatology, Merlin Park Hospital (Galway University Hospital), Renmore, Galway, Ireland
  2. 2NIHR-Leeds Musculoskeletal Biomedical Research Unit (NIHR-LMBRU), Leeds Institute of Molecular Medicine (LIMM), St James's University Hospital, University of Leeds, Leeds, UK
  1. Correspondence to N Quillinan, Department of Rheumatology, Merlin Park Hospital (Galway University Hospital), Renmore, Galway, Ireland; niamhq{at}gmail.com

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TNF-receptor-associated periodic syndrome (TRAPS) is an autoinflammatory condition and is the most common autosomal dominant hereditary periodic fever. Autoinflammatory syndromes are distinguished from autoimmune diseases by the fact that their disease immunopathology is largely played out by cells and molecules of the innate immune system.1 2

The pathogenesis of TRAPS is still not completely understood and treatment options are extremely limited. Prednisolone improves symptoms during attacks but the effect often wanes and patients may require increasing doses. Etanercept, a TNFR2 fusion protein, is reported to have a beneficial effect on many patients with TRAPS,3,,6 whereas infliximab is not generally effective in most cases of TRAPS and may cause an overwhelming flare but occasional benefit has also been described.7,,9 Anakinra, …

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Footnotes

  • Patient consent Obtained.

  • Ethics approval This study was conducted with the approval of the Galway University Hospitals Research Ethics Committee.

  • Provenance and peer review Not commissioned; externally peer reviewed.