POS0189 CANAKINUMAB AS 1ST OR 2ND BIOLOGIC IN SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS – DATA FROM THE BiKeR REGISTRY

Background: Canakinumab is approved for the treatment of systemic juvenile idiopathic arthritis (sJIA) older than 2 years of age. Real-world data are rare. Objectives: The aim of the German Biologics Registry (BiKeR) is the surveillance of JIA patients exposed to biologics including Canakinumab. Methods: Baseline demographics and disease activity parameters were documented. Efficacy was determined using the JADAS and the proposed criteria for inactive disease 1. Safety assessments were based on reports of adverse events (AE). All reports have been coded according to MedDRA ®. Results: 79/345 sJIA patients followed by the BIKER registry were treated with Canakinumab. In 39 patients, Canakinumab was used as first biologic, while 40 patients previously used other biologics (27 were pretreated with one, 12 with two and 2 with one biologic including pre-exposure to Anakinra in 18 patients. Concomitant treatment with steroids and/or methotrexate were given to 29 (37%) and 13 (16%) patients. Patients receiving Canakinumab first line were older (6.13.2-11.1 compared to 4.6 2.5-7.6 years) years but have a shorter disease duration (0.3 0.1-0.7 years compared to 1.1 0.5-5.1 years (median IQR). First line Canakinumab receivers had more frequently active systemic features (28 72% compared to 11 31% second-line users) and more often active arthritis (22 56% compared to 14 39%) while the mean number of active joints was comparable (1.9 +/12.8 vs. 1.9±3.6). First-line Canakinumab receivers showed a significantly higher disease activity reflected by a higher physicians global/parent global/CRP and sJADAS (16.7±8.0 compared to 9.8±8.1) and a higher mean number of active systemic features (2.1±1.3 compared to 0.9±0.9). In the first line cohort, the sJADAS (mean± SD) decreased from 16.7±8.7 to 2.0±3.5 at month 3 and remained low while in the preexposed cohort, the sJADAS decreased from 9.8±8.1 to 2.4±5.5 at month 3. Inactive disease defined as a sJADAS ≤2.9 (ref. 1) was reached at month 3, 6, 12, 18, 24 by 85%/87%/77%/67%/and 100% in first-line Canakinumab patients and by 87%/75%/84%/82% and 79% in biologics pre-exposed patients. A correlation was found between the disease duration before start of Canakinumab and the sJADAS10 at month 6(CC 0.31; p=0.019) and at month 12 (CC 0.246; p=0.06). 197 adverse events (AE) were reported in 57/79 (72%) within 167 patient-years (PY) of exposure to Canakinumab (118 events/100PY). Of these, 33 qualified as serious adverse events (SAE) (20/100PY). Adverse Events of Special Interest were serious and medically important infection (n=7 (4 pneumonias)), cytopenia (n=5), macrophage activation syndrome (n=6), hypersensitivity (n=2; DRESS, urticaria), evolving autoimmune diseases (2; psoriasis and type 1 diabetes mellitus) as well as each one thrombotic event, suicidality and overdose. There were no opportunistic infections, intestinal perforation, bleeding, malignancy, or death. No sJIA associated interstitial lung disease was reported. A total of 58 patients (73%) discontinued treatment, 10 (13%) due to lack or efficacy, 43 (54%) due to remission and 2 (3%) because of intolerance and 3 (4%) for other reasons. Conclusion: Canakinumab demonstrated comparable efficacy if used as first line or second line biologic for treatment of sJIA while a shorter disease duration was associated with a lower sJADAS at month 6 and 12. Safety was comparable and consistent with the overall AE profile of Canakinumab in paediatric patients. MAS as an JIA-associated feature occurred in an expected number of sJIA patients. Infections were the most frequent AE and seven medically important serious infections were reported. No new safety signals specific to the paediatric population were identified for Canakinumab; the risk profile of Canakinumab remains positive for the approved paediatric indication sJIA. REFERENCES: 1 Rosina S et al. Defining Cutoffs for Disease Activity States in Systemic Juvenile Idiopathic Arthritis Based on the Systemic Juvenile Arthritis Disease Activity Score abstract. Arthritis Rheumatol. 2023; 75 (suppl 9). Acknowledgements: NIL. Disclosure of Interests: Gerd Horneff MSD, Roche, Pfizer, Toni Hospach: None declared, Tilmann Kallinich: None declared, Frank Dressler: None declared, Frank Weller-Heinemann: None declared, Markus Hufnagel: None declared, Ariane Klein: None declared.