OP0042 LONG-TERM EFFICACY AND SAFETY OF CANAKINUMAB IN PATIENTS WITH FAMILIAL MEDITERRANEAN FEVER (FMF) - INTERIM ANALYSIS OF THE RELIANCE REGISTRY

Background Familial Mediterranean Fever (FMF) is a chronic disease characterized by recurrent attacks of fever as well serositis and bears the risk serious complications (e. g. amyloidosis). Treatment FMF according to EULAR aims control acute subclinical inflammation improve patient´s quality life 1 . Clinical data indicate that inhibition interleukin-1? with canakinumab (CAN) effective in controlling preventing flares patients 2 Objectives The present study explores long-term efficacy safety routine clinical practice conditions pediatric (age ?2 years) adult patients. Methods RELIANCE prospective, non-interventional, multi-center, observational based Germany 3-year follow-up period. Patients clinically confirmed diagnosis who routinely receive are enrolled order evaluate effectiveness canakinumab. Disease activity remission physicians´ assessment, activity, fatigue impact on social patients’ inflammatory markers AIDAI (Auto-Inflammatory Diseases Activity Index) score were recorded at baseline assessed 6-monthly intervals within observation period study. Results This interim analysis (N=74) December 2021 includes 6- 24-month data. Mean age this cohort was 25 years (2?61 proportion female 51 % (N=38). At baseline, median duration prior CAN treatment 1.0 (0?6 years). month 24, physician ratings report around 63% patient-reported (mean PPA) decreased from moderate (3.0) low (2.6) during Other parameters also (Table 1). A total 18 adverse events reported, which (1 case tonsillectomy tachycardia) classified drug - related. Table 1. Baseline characteristics 4 th 12 months 24 Number patients, N 74 46 (%) days absent work/school last 6 (8) 11 (24) 9 (38) (physician assessment) 22 (45) 23 (72) (63) Patient’s assessment current activity; 0–10, (min; max) 2.0 (0; 10) 7) fatigue; 5.0 4.0 without impairment 27 (50) 28 (80) 8 (67) CRP (mg/dl) | SAA ESR (mm/h); 0.2 0.7 8.0 0.5 6.0 disease-related symptoms inclusion into 68 (93) 14 (29) (25) 3 (16) Abdominal pain 67 (92) 20 (41) 10 (31) (21) Thoracic 45 (62) 5 (10) (9) (5) Headache 34 (47) (22) 7 (26) Myalgia (32) (12) (13) (11) Arthralgia/arthritis 39 (54) 16 (33) (28) Dermal (urticarial, maculopapulose) 15 0 (0) SAE Incidence rate # per 100 patient All types 14.03 SADR 1.56 = number * 36,525 / sum (=46,848). CRP, c-reactive protein; ESR, erythrocyte sedimentation rate; n. a., not annotated; SAA, serum amyloid A; SADR, reaction; SAE, events. Conclusion Interim study, longest running real-life registry confirm treatment. References [1]Ozen S, et al. recommendations for management familial fever. Ann Rheum Dis 2016;75:644–651. doi:10.1136/annrheumdis-2015-208690 [2]De Benedetti F, Canakinumab autoinflammatory syndromes. Engl J Med 2018;378:1908–19. Disclosure Interests Jörg Henes Consultant of: Novartis, AbbVie, Sobi, Roche, Janssen, Boehringer-Ingelheim, Grant/research support from: J. B. Kuemmerle-Deschner Tobias Krickau Speakers bureau: Tilmann Kallinich Frank Dressler Abbvie, Mylan, Pfizer, Gerd Horneff Bayer, Chugai, Merck Sharp & Dohme, Florian Meier Ivan Foeldvari Hexal, Medac, Weller-Heinemann: None declared, Birgit Kortus-Goetze Markus Hufnagel Jürgen Rech Biogen, BMS, GSK, Lilly, MSD; Sanofi, UCB, MSD, Prasad Oommen Julia Weber-Arden Employee Norbert Blank Actelion, Sobi.