OR10-005 - Treatment responses in TRAPS: Eurofever/ Eurotraps

Results In total there was treatment data on 113 patients with 45 different PSV of TNFRSF1A. Patients came from 14 countries and 94.5% were of European Caucasian ancestry. 16 patients had only received symptomatic treatment. Of 48 patients given steroids only with attacks 20 (42%) reported complete success (CR) in terminating acute attacks but 38 (79%) were either converted to biologic therapy or had them added to improve disease control. Of 22 patients on maintenance steroids 6 (27%) reported complete attack prevention but 14 (64%) were converted to biologic therapy. 37 patients received etanercept (in the 19 where data was available for a median of 51 months). 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 (range 189 months).