POS1380 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 autoinflammatory condition characterized by severe systemic and organ inflammation. In phase 3 pivotal trial ( CLUSTER study), TRAPS patients have been successfully treated with the interleukin-1β inhibitor canakinumab. 45% of reached clinical remission after 16 weeks (primary endpoint) 1 . Canakinumab has approved applied for treatment since 2017 2 Objectives: The present study explores long-term efficacy safety canakinumab under routine practice conditions in pediatric (age ≥2 years) adult patients. Methods: RELIANCE prospective, non-interventional, multi-center, observational based Germany 3-year follow-up period. Patients clinically confirmed diagnoses TRAPS, CAPS, FMF or HIDS/MKD who routinely receive are enrolled order to evaluate standard conditions. Disease activity physician assessment, disease fatigue patient days absent from school/work due indication, inflammatory markers, AIDAI (Auto-Inflammatory Diseases Activity Index) score were assessed at baseline 6-monthly intervals. Results: interim analysis December 2020 includes (N=16, including atypical TRAPS) preliminary 18-month data. Mean age this cohort was 23 years (3−43 median duration prior CAN 1.0 year (0−4 years). Physician assessment indicated 60-80% laboratory parameters within normal range. control showed no major changes regarding analyzed (Table 1, Figure 1). Of three serious adverse events reported none classified as drug-related. Conclusion: Preliminary 18 month data available indicate stable treatment. References: [1]De Benedetti F, et al. recurrent fever syndromes. N Engl J Med 2018;378:1908–19. [2]Ilaris, INN-canakinumab (europa.eu) Table 1. Baseline characteristics 6 months 12 Number patients, 13 10 Median age, (min; max) (3; 43) 17 (4; 38) 25 Females (%) 11 (69) 9 7 (70) (50) therapy baseline, (0; 4) 1.5 2) (physician assessment) (60.0) (81.8) (77.8) 4 (80.0) Global Assessment, percentage absent/mild-moderate/severe rating 40 / 53 0 82 44 80 20 Patient current activity; 0–10, 5) 6) 0.0 3) fatigue; 2.0 8) 7) 2,5 4.0 without impairment social life 5 (63) (33) (60) work/school during last 8 (39) (56) CRP, (mg/dl) 0.1 SAA, 0.5 0.4 0.3 ESR, (mm/h) 7.0 5.0 SAE Incidence rate per 100 Total 14.7 Circulatory collapse (non fatal) 4.9 Dizziness Headache c-reactive protein; ESR , erythrocyte sedimentation rate; serum amyloid A; SAE, event Disclosure Interests: Norbert Blank Consultant of: Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, Roche, Grant/research support from: Jörg Henes AbbVie, Janssen, Tilmann Kallinich Prasad Oommen Catharina Schuetz: None declared, Michael Borte Shire, Julia Weber-Arden Employee J. B. Kuemmerle-Deschner Sobi