POS0221 LONG-TERM EFFICACY AND SAFETY OF CANAKINUMAB IN PATIENTS WITH TRAPS (TUMOR NECROSIS FACTOR RECEPTOR-ASSOCIATED PERIODIC SYNDROME) – INTERIM ANALYSIS OF THE RELIANCE REGISTRY

Background Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a rare hereditary autoinflammatory disease characterized by fever as well severe systemic and organ inflammation. Successful treatment was achieved with the interleukin-1β inhibitor canakinumab (CAN) in pivotal phase 3 study, which 45% of patients reached clinical remission after 16 weeks (primary endpoint) 1 . CAN has been approved applied for TRAPS since 2017 2 Objectives The present study explores long-term efficacy safety under routine practice conditions pediatric (age ≥2 years) adult patients. Methods RELIANCE prospective, non-interventional, multi-center, observational based Germany. Patients clinically confirmed diagnosis who routinely receive are enrolled order to evaluate standard at baseline 6-monthly intervals. Results interim analysis December 2021 includes (N=19, patient atypical TRAPS) preliminary 24-month data. Of these patients, N=12 (63%) were females median age years (3–43 years). Preliminary results indicate stable physicians´ assessment laboratory parameters. Disease control patients´ showed no major changes over time (Table 1). In total, N=7 adverse drug reactions where observed, none classified severe. Table 1. Baseline characteristics data TRAPS. Baseline(N=19) 12 months(N=15) 24 months(N=8) Number (%) (physician assessment) 9 (53) 11 (79) 4 (67) Physician activity, percentage absent/mild-moderate/severe rating 35 / 59 0 57 36 7 67 33 Patient´s current activity; 0–10, (min; max) 1.5 (0; 8) 1.0 6) 7) fatigue; without impairment social life 5 (56) (44) (100) days absent from work/school during last 6 months 8 (40) (14) CRP, (mg/dl) 0.2 0.1 SAA, 0.5 0.4 ESR, (mm/h) 7.0 5.5 6.0 SAE events Incidence rate # per 100 All types 24.2 SADR 0.0 c-reactive protein; serum amyloid A; erythrocyte sedimentation rate; #Numbers/percentage do not sum up N=19/100%, due unknown weight some Conclusion available study. Further end-of-study will be analyzed assess dosing effects References [1]De Benedetti F, et al. Canakinumab recurrent syndromes. N Engl J Med 2018;378:1908–19 [2]Ilaris, INN-canakinumab (europa.eu) Disclosure Interests Norbert Blank Consultant of: Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, Roche, Grant/research support from: Catharina Schuetz: None declared, Jörg Henes AbbVie, Janssen, Tilmann Kallinich Prasad Oommen Michael Borte Shire, Markus Hufnagel Ales Janda: Julia Weber-Arden Employee J. B. Kuemmerle-Deschner Sobi