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

Indian Pediatr

Pediatric Rheumatology Clinic, Jaslok Hospital and Research Center, Mumbai, Maharashtra, India. Correspondence to: Dr RP Khubchandani, Consultant Pediatrician, Jaslok Hospital and Breach Candy Hospital, Mumbai, Maharashtra, India.

Published: January 2014

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.

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Source
http://dx.doi.org/10.1007/s13312-014-0313-3DOI Listing

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