Skip to main content
Log in

Modified regimen of etanercept for tumor necrosis factor receptor associated periodic syndrome (TRAPS) like illness

  • Case Reports
  • Published:
Indian Pediatrics Aims and scope Submit manuscript

Abstract

Background

TRAPS, an autosomal dominant autoinflammatory disorder occurs due to mutations of the TNFRSF1A gene. Mutation negative TRAPS (TRAPS like illness) is also known. Anti TNF molecules (etanercept) is the mainstay of therapy.

Case Characteristics

A 11-year-old boy with a 5 year clinical profile indicative of a TRAPS like illness and with negative mutation studies is described. He has been followed up for nearly 2 years after starting etanercept.

Outcome

He had sustained response to etanercept which has subsequently been titrated (0.4 mg/kg subcutaneously every 23–24 days) to keep him symptom free.

Message

Mutation negative cases of TRAPS can be diagnosed with a high index of suspicion. Treatment with etanercept is expensive but possibly intervals between doses could be titrated to reduce cost.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Chen YJ, Yu HH, Yang YH, Lau YL, Lee WI, Chiang BL. Recurrent abdominal pain as the presentation of tumor necrosis factor receptor-associated periodic syndrome (TRAPS) in an Asian girl: A case report and review of the literature. J Microbiol Immunol Infect. 2012; Aug 23. [E-.pub ahead of print].

    Google Scholar 

  2. Cantarini L, Lucherini OM, Cimaz R, Rigante D, Baldari CT, Laghi Pasini F, et al. Typical and severe tumor necrosis factor receptor-associated periodic syndrome in the absence of mutations in the TNFRSF1A gene: a case series. Rheumatol Int. 2012;32:4015–4018.

    Article  CAS  PubMed  Google Scholar 

  3. Baron L, Athreya B, Kastner D. Periodic fever syndromes and other autoinflammatory disorders. In: Cassidy JT, Petty RE, Laxer RM, Lindsley CB, editors. Textbook of Pediatric Rheumatology. 6th ed. Philadelphia: Saunders Elsevier 2011. P. 647.

    Google Scholar 

  4. Bodar EJ, Dreanth JPH, van der Meer JWM, Simon A. Dysregulation of innate immunity, hereditary periodic fever syndromes. BrJ Hematol. 2009;144:279–302.

    Article  CAS  Google Scholar 

  5. Aganna E, Hammond L, Hawkins PN, Aldea A, McKee SA, van Amstel HK, et al. Heterogeneity among patients with tumor necrosis factor receptor-associated periodic syndrome phenotypes. Arthritis Rheum. 2003;48:2632–2644.

    Article  CAS  PubMed  Google Scholar 

  6. Jacobelli S, André M, Alexandra JF, Dodé C, Papo T. Failure of anti-TNF therapy in TNF receptor 1-associated periodic syndrome (TRAPS) Rheumatology. 2007;46:1211–1212.

    Article  CAS  PubMed  Google Scholar 

  7. Drewe E, McDermott EM, Powell PT, Isaacs JD, Powell RJ. 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. Rheumatol. 2003;42:235–239.

    Article  CAS  Google Scholar 

  8. Bulua AC, Mogul DB, Aksentijevich I, Singh H, He DY, Muenz LR, et al. Efficacy of etanercept in the tumor necrosis factor receptor-associated periodic syndrome: a prospective, open-label, dose-escalation study. Arthritis Rheum. 2012;64:908–913.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to R. P. Khubchandani.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dhanrajani, A., Khubchandani, R.P. Modified regimen of etanercept for tumor necrosis factor receptor associated periodic syndrome (TRAPS) like illness. Indian Pediatr 51, 55–57 (2014). https://doi.org/10.1007/s13312-014-0313-3

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s13312-014-0313-3

Keywords

Navigation