Date: Saturday, November 6, 2021
Session Title: Miscellaneous Rheumatic & Inflammatory Diseases Poster I (0183–0209)
Session Type: Poster Session A
Session Time: 8:30AM-10:30AM
Background/Purpose: The IL-1 inhibitor canakinumab (CAN) induces rapid remission of symptoms of CAPS (Cryopyrin-associated periodic syndromes), a monogenic autoinflammatory disease with severe systemic inflammation, both in clinical trials and in routine practice.The aim of the present study is to investigate the long-term efficacy and safety of CAN in pediatric (≥ 2 years) and adult patients with CAPS (incl. Muckle-Wells syndrome [MWS], familial cold-induced autoinflammatory syndrome [FCAS], and neonatal onset inflammatory systemic disease [NOMID]/chronic infantile neurologic-cutaneous-articular syndrome [CINCA]) in relation to weight-dependent CAN dosing under routine clinical practice conditions.
Methods: The prospective, non-interventional, observational RELIANCE study with 3-year follow-up includes patients with clinically confirmed CAPS diagnosis who routinely receive CAN. Clinical data, physician assessment, and patient-reported outcomes are evaluated at 6-month visits from baseline.
Results: The interim analysis included data from 91 CAPS patients (50% females; 14 [15%] NOMID/CINCA subtypes) enrolled by December 2020. At baseline, median age was 20.5 years and median duration of prior CAN treatment was 6 years. According to physician assessment, 66% of patients achieved disease remission with increasing absence of disease activity according to PGA (physician global assessment, 50-60%). Patients reported stable, low levels of disease activity, fatigue, and Auto-Inflammatory Diseases Activity Index (AIDAI) scores. CAPS affected the social life of 50% of patients and 50% reported days of absence from school/work. At baseline, 2% of patients received less than the standard dose* CAN (SD; < 87.5% of SD), 49% received standard dose CAN (SD CAN; 150 mg and 2mg/kg respectively per 8 weeks) and another 49% received higher than SD CAN ( >112.5% of SD). At 30 months, 82% of patients received higher than SD CAN. The laboratory parameters as well as physicians´ and patients´ rating of disease activity remained stable over time, showing sustained remission and disease control in all dosing groups (Table 1).
Treatment-related severe adverse events (SAE) occurred in 8 patients, none of them under standard dosing (in 1 patient: less than SD, in 7 patients more than SD).
Conclusion: The 30-month interim analysis of the RELIANCE study shows that long-term treatment with CAN is safe and effective in patients with CAPS. A clear trend towards updosing to more than SD CAN could be observed.
To cite this abstract in AMA style:Kuemmerle-Deschner J, Kortus-Goetze B, Oommen P, Janda A, Rech J, Kallinich T, Weller-Heinemann F, Horneff G, Foeldvari I, Schuetz C, Borte M, Braner A, Weber-Arden J, Blank N. Influence of Canakinumab Dosing on Long-term Efficacy and Safety in Patients with Cryopyrin-associated Periodic Syndromes (CAPS) – 30-months Interim Analysis of the RELIANCE Registry [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/influence-of-canakinumab-dosing-on-long-term-efficacy-and-safety-in-patients-with-cryopyrin-associated-periodic-syndromes-caps-30-months-interim-analysis-of-the-reliance-registry/. Accessed June 1, 2023.
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ACR Meeting Abstracts - https://acrabstracts.org/abstract/influence-of-canakinumab-dosing-on-long-term-efficacy-and-safety-in-patients-with-cryopyrin-associated-periodic-syndromes-caps-30-months-interim-analysis-of-the-reliance-registry/