Higher Rates of Disease Control During the Coronavirus Pandemic in Pediatric Patients with Autoinflammatory Periodic Diseases on Canakinumab Treatment – Interim Data from the RELIANCE Registry

Gerd Horneff¹, Norbert Blank², Jasmin B. Kuemmerle-Deschner³, Joerg Henes⁴, Birgit Kortus-Goetze⁵, Prasad T. Oommen⁶, Anne Pankow⁷, Tobias Krickau⁸, Catharina Schuetz⁹, Ivan Foeldvari¹⁰, Juergen Rech¹¹, Frank Weller-Heinemann¹², Ales Janda¹³, Markus Hufnagel¹⁴, Florian M. Meier¹⁵, Frank Dressler¹⁶, Michael Borte¹⁷, Ioana Andreica¹⁸, Peter Wasiliew¹⁹, Michael Fiene²⁰, Daniel Windschall²¹, Martin Krusche²², Tania Kuempfel²³, Julia Weber-Arden²⁴ and Tilmann Kallinich²⁵, ¹Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, ²University Hospital Heidelberg, Heidelberg, Germany, ³med.uni-tuebingen, Tübingen, Germany, ⁴University Hospital Tuebingen, Tuebingen, Germany, ⁵Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, ⁶Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁷Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, ⁸Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, ⁹Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, ¹⁰Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹¹University Clinic Erlangen, Erlangen, Germany, ¹²Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, ¹³Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, ¹⁴Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁵Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, ¹⁶Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, ¹⁷Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, ¹⁸Rheumazentrum Ruhrgebiet Herne, Herne, Germany, ¹⁹Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ²⁰Rheumatology Center Greifswald, Greifswald, Germany, ²¹Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, ²²UKE, Hamburg, Germany, ²³Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, ²⁴Novartis Innovative Medicines, Nuernberg, Germany, ²⁵Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany

Meeting: ACR Convergence 2023

Keywords: Autoinflammatory diseases, Disease Activity, Innate Immunity Rheumatic Disease, Periodic Disease

Session Information

Date: Tuesday, November 14, 2023

Title: (1913–1944) Miscellaneous Rheumatic & Inflammatory Diseases Poster III

Session Type: Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Pediatric patients with autoinflammatory diseases (AID) on Canakinumab (CAN) therapy have been affected by the coronavirus pandemic including SARS-CoV-2 infection, SARS-CoV-2 vaccination, and AID disease management. In the RELIANCE registry, severity of SARS-CoV-2 infection, safety of SARS-CoV-2 vaccination and comparison of disease management before and during pandemic in pediatric patients with cryopyrin-associated periodic syndromes (CAPS), familial Mediterranean fever (FMF), hyper-IgD syndrome/mevalonate kinase deficiency (HIDS/MKD) and tumor necrosis factor receptor-associated periodic syndrome (TRAPS) on CAN therapy was investigated in clinical practice.

Methods: The RELIANCE registry is a prospective, non-interventional, observational study in Germany enrolling pediatric (age ≥2 years) and adult patients with a clinically confirmed diagnosis of AID who routinely receive CAN. Efficacy and safety parameters are recorded at baseline and assessed at 6-month intervals.

Results: The present interim analysis includes data from n=101 pediatric patients with AID enrolled in the RELIANCE registry between October 2017 and December 2022. The median duration of CAN treatment before and during the study was 3.7 years (0-13.5 years).

During the study, 29 SARS-CoV-2 infections were reported for n=27 pediatric patients. 22 infections caused mild symptoms and 7 infections caused moderate symptoms. N=25 (24.8 %) of pediatric patients received at least one SARS-CoV-2 vaccination (15 Comirnaty, 13 not specified). Vaccination reactions (not further specified) were reported in n=3 patients. No reactions were classified as serious.

During the COVID-19 pandemic in 2020 and 2021, only 83 % of regular patient visits were performed (Fig. 1). In addition, patients received slightly lower CAN dosing (20% standard dose CAN [SD], 17% lower than SD, and 63% higher than SD compared to 24% SD, 10% lower than SD, and 66% higher than SD before pandemic (Fig. 2). However, disease activity by patient rating (VAS score 1-10) improved from 3 before to 2 during the pandemic. By physician global assessment, the proportion of pediatric patients with no disease activity arose from 20% before to 45% during the pandemic (Fig. 3). In contrast: no major changes in disease activity were documented in adult patients.

Conclusion: Pediatric AID patients under long-term Canakinumab treatment experienced improved disease control during the Coronavirus pandemic.

Figure 1: Planned and performed physician visits of pediatric patients before and during coronavirus pandemic.

Figure 2: Canakinumab dose categories before and during coronavirus pandemic.

Figure 3: Physician assessment of disease activity before and during coronavirus pandemic.

Disclosures: G. Horneff: GSK, 6, Janssen, 6, MSD, 5, 6, Novartis, 5, 6, Pfizer, 5, 6, Roche, 5, 6, Sanofi, 6, Sobi, 6; N. Blank: Novartis, Sobi, 5, Novartis, Sobi, Lilly, Pfizer, Abbvie, BMS, MSD, Actelion, UCB, Boehringer-Ingelheim, Roche, 2; J. Kuemmerle-Deschner: Novartis, AbbVie, Sobi, 2, 5; J. Henes: AbbVie/Abbott, 2, 6, Boehringer-Ingelheim, 2, 6, Bristol-Myers Squibb(BMS), 2, 6, GlaxoSmithKlein(GSK), 2, 6, Janssen, 2, 6, Novartis, 2, 6, Pfizer, 2, 6, UCB, 2, 6; B. Kortus-Goetze: Novartis, 2; P. Oommen: Novartis, 5; A. Pankow: None; T. Krickau: Novartis, 2, 5, 6; C. Schuetz: Novartis, 5; I. Foeldvari: Novartis, 2; J. Rech: AbbVie, Biogen, BMS, Chugai, GSK, Janssen, Lilly, MSD; Mylan, Novartis, Roche, Sanofi, Sobi, UCB, 2, 6, Novartis, Sobi, 5; F. Weller-Heinemann: None; A. Janda: None; M. Hufnagel: Novartis, 5; F. Meier: Novartis, 6; F. Dressler: Abbvie, Mylan, Novartis, Pfizer, 2, Novartis, 5; M. Borte: Pfizer, Shire, 5; I. Andreica: AbbVie/Abbott, 1, 6, Amgen, 1, 6, AstraZeneca, 1, 6, Chugai, 6, Novartis, 1, 6, Sobi, 1, 6, UCB, 1, 6; P. Wasiliew: None; M. Fiene: None; D. Windschall: None; M. Krusche: Chugai, 2, 6; T. Kuempfel: None; J. Weber-Arden: Novartis, 3; T. Kallinich: Roche, 6.

To cite this abstract in AMA style:

Horneff G, Blank N, Kuemmerle-Deschner J, Henes J, Kortus-Goetze B, Oommen P, Pankow A, Krickau T, Schuetz C, Foeldvari I, Rech J, Weller-Heinemann F, Janda A, Hufnagel M, Meier F, Dressler F, Borte M, Andreica I, Wasiliew P, Fiene M, Windschall D, Krusche M, Kuempfel T, Weber-Arden J, Kallinich T. Higher Rates of Disease Control During the Coronavirus Pandemic in Pediatric Patients with Autoinflammatory Periodic Diseases on Canakinumab Treatment – Interim Data from the RELIANCE Registry [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/higher-rates-of-disease-control-during-the-coronavirus-pandemic-in-pediatric-patients-with-autoinflammatory-periodic-diseases-on-canakinumab-treatment-interim-data-from-the-reliance-registry/. Accessed .

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