PW02-003 - Efficacy of anakinra in etanercept-resistant TRAPS

Ak Ombrello,A Jones,Ks Barron,Dl Kastner,Pm Hoffmann

doi:10.1186/1546-0096-11-s1-a143

Abstract

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autoinflammatory disease inherited in an autosomal dominant fashion. TRAPS develops secondary to mutations in TNFRSF1A. Associated symptoms include periodic attacks of peritonitis, constipation, arthritis in large joints, arthralgia, migratory rash with underlying myalgia, periorbital edema, conjunctivitis, splenomegaly, and increased risk for AA amyloidosis. Typically, attacks last from days to weeks. The common treatment modalities are corticosteroids, the p75 TNFR:Fc fusion protein, etanercept, and IL-1 antagonists. Recent studies suggest that multiple cytokines are involved in the pathogenesis of TRAPS. To date, there are limited data comparing the efficacy of etanercept and IL-1 inhibitors in TRAPS.

Highlights

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autoinflammatory disease inherited in an autosomal dominant fashion
There are limited data comparing the efficacy of etanercept and IL-1 inhibitors in TRAPS
Our findings indicate that in some patients, anakinra is superior to etanercept for the treatment of TRAPS

Summary

Introduction

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autoinflammatory disease inherited in an autosomal dominant fashion. TRAPS develops secondary to mutations in TNFRSF1A. Associated symptoms include periodic attacks of peritonitis, constipation, arthritis in large joints, arthralgia, migratory rash with underlying myalgia, periorbital edema, conjunctivitis, splenomegaly, and increased risk for AA amyloidosis. The common treatment modalities are corticosteroids, the p75 TNFR: Fc fusion protein, etanercept, and IL-1 antagonists. Recent studies suggest that multiple cytokines are involved in the pathogenesis of TRAPS. There are limited data comparing the efficacy of etanercept and IL-1 inhibitors in TRAPS

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Results