Long-term Safety of Canakinumab in Patients with Autoinflammatory Periodic Fever Syndromes - Interim Analysis of the RELIANCE Registry

Jasmin Kuemmerle-Deschner¹, Jorg Henes², Birgit Kortus-Goetze³, Tilmann Kallinich⁴, Prasad T. Oommen⁵, Juergen Rech⁶, Frank Weller-Heinemann⁷, Gerd Horneff⁸, Ivan Foeldvari⁹, Aleš Janda¹⁰, Catharina Schuetz¹¹, Frank Dressler¹², Michael Borte¹³, Markus Hufnagel¹⁴, Axel Braner¹⁵, Florian Meier¹⁶, Michael Fiene¹⁷, Julia Weber-Arden¹⁸ and Norbert Blank¹⁹, ¹Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, ²University Hospital Tuebingen, Tuebingen, Germany, ³Division of Nephrology, University of Marburg, Marburg, Germany, ⁴Charité University Medicine, Nuremberg, Germany, ⁵Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁶Friedrich-Alexander University (FAU) Erlangen-Nuernberg and Universitaetsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology, Germany, Erlangen, Germany, ⁷Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁸Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁹Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹⁰Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ¹¹Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ¹²Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ¹³ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Leipzig, Germany, ¹⁴Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁵Department of Medicine II, Rheumatology, University Hospital Frankfurt, Germany, Frankfurt a.M., Germany, ¹⁶1 Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany 2 Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt am Main, Germany, Frankfurt a.M., Germany, ¹⁷Department of Internal Medicine, District Hospital Demmin, Demmin, Germany, ¹⁸Novartis Pharma GmbH, Nuremberg, Germany, ¹⁹Rheumatology, University Hospital Heidelberg, Eppelheim, Germany

Meeting: ACR Convergence 2021

Keywords: Autoinflammatory diseases, IL-1ß blockade, Inflammation, registry, Safety

Session Information

Date: Saturday, November 6, 2021

Title: Miscellaneous Rheumatic & Inflammatory Diseases Poster I (0183–0209)

Session Type: Poster Session A

Session Time: 8:30AM-10:30AM

Background/Purpose: Autoinflammatory periodic fever syndromes (PFS) are characterized by severe systemic and organ inflammation. In clinical trials, successful treatment was achieved with the interleukin-1β inhibitor canakinumab (CAN).

The present study explores the long-term efficacy and safety of CAN in routine clinical practice conditions in pediatric (age ≥2 years) and adult patients with CAPS (cryopyrin-associated periodic syndromes), FMF (familial Mediterranean fever), TRAPS (tumor necrosis factor receptor-associated periodic syndrome) and HIDS/MKD (hyperimmunoglobulinemia D syndrome/mevalonate kinase deficiency).

Methods: RELIANCE is a prospective, non-interventional, observational study based in Germany. Patients with clinically confirmed diagnoses of PFS routinely receiving CAN are enrolled. Besides efficacy parameters regarding disease activity and remission, safety parameters were recorded at baseline and assessed at 6-monthly intervals.

Results: Here we present the interim analysis of 168 patients with PFS enrolled in the RELIANCE Registry between October 2017 and December 2020. Mean age in this cohort was 24.7 years (2-79 years) and the proportion of female patients was 51 %. At baseline, median duration of prior CAN treatment was 3 years (0-12 years).

A total of 101 patients (60%) experienced any adverse event (AE) and 22 patients (13%) were affected by severe adverse events (SAE). In 9 patients (5%) SAE were classified as drug related. Of 489 AE, 53 were severe and a total of 21 SAE were classified as treatment-related (table). Overall, 13 AE comprised upper respiratory tract infections (ARI).

Conclusion: The interim data from the RELIANCE study, the longest running real-life canakinumab registry for autoinflammatory periodic fever syndromes, confirm safety of long-term canakinumab treatment across the entire study population.

Table: Overview of the CAN safety data of the RELIANCE study across all study indications (N=168 patients).
‡IR, incidence rate per 100 patient years; AE, adverse event; SAE, severe adverse event, SADR, severe adverse drug reaction

Disclosures: J. Kuemmerle-Deschner, Novartis, 1, 2, 5, 6, SOBI, 1, 2, 5, 6; J. Henes, Roche Pharma, 1, 5, 6, ABBVIE, 6, Novartis, 5, 6, SOBI, 5, 6, LILLY, 6, Pfizer, 5, 6, BMS, 6, Janssen, 6, UCB, 6, Boehringer-Ingelheim, 6; B. Kortus-Goetze, Novartis, 2; T. Kallinich, None; P. Oommen, Novartis, 5; J. Rech, Novartis, 2, 5, 6, SOBI, 2, 5, 6, AbbVie, 2, 6, Biogen, 2, 6, BMS, 2, 6, Chugai, 2, 6, GSK, 2, 6, Janssen, 2, 6, Lilly, 2, 6, MSD, 2, 6, Mylan, 2, 6, Roche, 2, 6, Sanofi, 2, 6, UCB, 2, 6; F. Weller-Heinemann, None; G. Horneff, Novartis, 5, 6, Janssen, 5, 6, Roche, 5, Eli-Lilly, 6, Glaxo Smith and Kline, 6, Pfizer, 6, Sobi, 6; I. Foeldvari, Novartis, 4; A. Janda, None; C. Schuetz, None; F. Dressler, Novartis, 2, 5, AbbVie, 2, Mylan, 2, Pfizer, 2; M. Borte, Shire, 5, Pfizer, 5; M. Hufnagel, None; A. Braner, Novartis, 2, SOBI, 2; F. Meier, Novartis, 6; M. Fiene, None; J. Weber-Arden, Novartis, 3; N. Blank, Novartis, 2, 5, SOBI, 2, 5, Lilly, 2, Pfizer, 2, AbbVie, 2, BMS, 2, MSD, 2, Actelion, 2, UCB, 2, Boehringer Ingelheim, 2, Roche, 2.

To cite this abstract in AMA style:

Kuemmerle-Deschner J, Henes J, Kortus-Goetze B, Kallinich T, Oommen P, Rech J, Weller-Heinemann F, Horneff G, Foeldvari I, Janda A, Schuetz C, Dressler F, Borte M, Hufnagel M, Braner A, Meier F, Fiene M, Weber-Arden J, Blank N. Long-term Safety of Canakinumab in Patients with Autoinflammatory Periodic Fever Syndromes – Interim Analysis of the RELIANCE Registry [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/long-term-safety-of-canakinumab-in-patients-with-autoinflammatory-periodic-fever-syndromes-interim-analysis-of-the-reliance-registry/. Accessed .

ACR Meeting Abstracts - https://acrabstracts.org/abstract/long-term-safety-of-canakinumab-in-patients-with-autoinflammatory-periodic-fever-syndromes-interim-analysis-of-the-reliance-registry/