Abstract

TRAPS is a rare lifelong disease. Optimal treatment is not established but tends to rely either on corticosteroids with risks of known short and long term side effects or anti cytokine agents which are expensive, and both types of agents lead to higher risk of infection.

Highlights

  • To analyze treatments used and their responses in patients with clinical TRAPS associated with a pathogenic sequence variants (PSV) in TNFRSF1A enrolled in the Eurofever/Eurotraps registry

  • Of 22 patients on maintenance steroids 6 (27%) reported complete attack prevention but 14 (64%) were converted to biologic therapy. 37 patients received etanercept. 9 patients had a CR and 26 a partial response (PR). 10 remain on etanercept

  • Of the 27 who discontinued etanercept inadequate disease response was sole or contributory reason for discontinuing etanercept in 21 and side effects in 9. 20 patients converted to anti IL-1 therapy. 38 patients received anakinra. 34 (89%) reported a CR and 4 a PR. 92% remain on anakinra with a median treatment duration date of 23 months

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Summary

Introduction

OR10-005 - Treatment responses in TRAPS: Eurofever/ Eurotraps Introduction TRAPS is a rare lifelong disease. Optimal treatment is not established but tends to rely either on corticosteroids with risks of known short and long term side effects or anti cytokine agents which are expensive, and both types of agents lead to higher risk of infection. Objectives To analyze treatments used and their responses in patients with clinical TRAPS associated with a pathogenic sequence variants (PSV) in TNFRSF1A enrolled in the Eurofever/Eurotraps registry.

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Results
Conclusion

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