Background Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with dysregulation of the innate immune system driven by cytokines. A major role is ascribed to interleukin-1β (IL-1β), supporting the autoinflammatory character of the disease and offering an effective blocking mechanism for treatment. Here we present clinical practice data from the German AID-registry for patients treated with IL-1 inhibition (IL-1i). Methods In 2009 a clinical and research consortium (AID-Net) was established, including an online AID-registry. Patients with documented sJIA diagnosis were identified. Data for this retrospective IL-1i study were recorded by 17 centers. Response to treatment was evaluated according to Wallace criteria and additionally by an own classifying clinical response system. Results In 6 years, 202 patients with confirmed sJIA were recorded in the AID-registry. Out of these, 111 children received therapy with Anakinra (ANA) ( n = 84, 39 f) and/or Canakinumab (CANA) ( n = 27, 15 f) at a median age of 8.7 y (range 0.6–19.1). During the first 12 months 75/111 (ANA 55, CANA 20) patients were evaluated according to Wallace criteria (achievement of inactive disease 28/55 and 17/20, remission over 6 months under medication 13/55 and 7/20 cases). Over the whole period of time, clinical response was preserved in the majority of patients (ANA 54/80, CANA 20/27). Arthritis mostly persisted in polyarticular (PA) courses. During treatment with IL-1i concomitant medication could be tapered in about 15%. IL-1i was discontinued in 59/111 patients. 45 (15) adverse events (AE)s in ANA (CANA) treated patients (19.7 (26.6) AE/100 ANA (CANA) exposure years, 95%CI: 14.4–26.4 (14.9–43.9)) were reported. Conclusion In a large cohort of sJIA patients from Germany, we can confirm an overall favorable clinical response to both available IL-1 blocking agents. IL-1i was well tolerated with acceptable safety and effectiveness in a real-life clinical setting.
Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry
E. Lainka,Melanie Baehr,Bernadette Raszka,J. Haas,B. Hügle,N. Fischer,D. Foell,C. Hinze,E. Weissbarth-Riedel,T. Kallinich,G. Horneff,D. Windschall,E. Lilienthal,T. Niehues,U. Neudorf,R. Berendes,R. Küster,P. Oommen,C. Rietschel,T. Lutz,F. Weller-Heinemann,K. Tenbrock,G. Heubner,J. Klotsche,H. Wittkowski
Published 2021 in Pediatric Rheumatology Online Journal
ABSTRACT
PUBLICATION RECORD
- Publication year
2021
- Venue
Pediatric Rheumatology Online Journal
- Publication date
2021-03-22
- Fields of study
Medicine
- Identifiers
- External record
- Source metadata
Semantic Scholar, PubMed
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