Prospective study of anti-tumour necrosis factor receptor superfamily 1B fusion protein, and case study of anti-tumour necrosis factor receptor superfamily 1A fusion protein, in tumour necrosis factor receptor associated periodic syndrome (TRAPS): clinical and laboratory findings in a series of seven patients

Rheumatology (Oxford). 2003 Feb;42(2):235-9. doi: 10.1093/rheumatology/keg070.

Abstract

Objective: To assess the effects prospectively of tumour necrosis factor (TNF) receptor superfamily (TNFRSF) fusion proteins TNFRSF1B (etanercept) and TNFRSF1A (p55TNFr-Ig) in patients with TNF receptor associated periodic syndrome (TRAPS).

Methods: Seven patients with a clinical and genetic diagnosis of TRAPS received subcutaneous etanercept for 24 weeks. One of these patients had previously received an intravenous infusion of p55TNFr-Ig. Therapeutic response was assessed by comparing corticosteroid requirement, acute-phase response and an established scoring system over 20 weeks, both on and off etanercept.

Results: Etanercept was well tolerated. The five corticosteroid-responsive patients required significantly less corticosteroids and demonstrated reductions in acute-phase reactants on etanercept. The two patients not requiring corticosteroids had small reductions in disease activity scores. The effect of p55TNFr-Ig in a single patient with TRAPS remains unclear.

Conclusions: Etanercept does not abolish inflammatory attacks but improves disease activity allowing corticosteroid reduction. Etanercept may be clinically useful in replacing or reducing steroid requirements in the treatment of TRAPS. A formal trial of etanercept to establish its role in clinical management is indicated.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Antigens, CD / therapeutic use
  • Antirheumatic Agents / therapeutic use*
  • C-Reactive Protein / metabolism
  • Child, Preschool
  • Drug Administration Schedule
  • Drug Therapy, Combination
  • Etanercept
  • Familial Mediterranean Fever / drug therapy*
  • Female
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunoglobulin G / therapeutic use*
  • Male
  • Middle Aged
  • Prospective Studies
  • Receptors, Tumor Necrosis Factor / therapeutic use*
  • Receptors, Tumor Necrosis Factor, Type I
  • Treatment Outcome

Substances

  • Antigens, CD
  • Antirheumatic Agents
  • Glucocorticoids
  • Immunoglobulin G
  • Receptors, Tumor Necrosis Factor
  • Receptors, Tumor Necrosis Factor, Type I
  • C-Reactive Protein
  • Etanercept