000K utf8 1100 2021$c2021-03-22 1500 eng 2050 urn:nbn:de:hbz:465-20240904-091407-2 2051 10.1186/s12969-021-00510-8 3000 Lainka, Elke 3010 Baehr, Melanie 3010 Berendes, Rainer 3010 Fischer, Nadine 3010 Foell, Dirk 3010 Haas, Johannes-Peter 3010 Heubner, Georg Leonhard 3010 Hinze, Claas 3010 Horneff, Gerd 3010 Hügle, Boris 3010 Kallinich, Tilmann 3010 Klotsche, Jens 3010 Küster, Rolf-Michael 3010 Lilienthal, Eggert 3010 Lutz, Thomas 3010 Neudorf, Ulrich 3010 Niehues, Tim 3010 Oommen, Prasad Thomas 3010 Raszka, Bernadette 3010 Rietschel, Christoph 3010 Tenbrock, Klaus 3010 Weissbarth-Riedel, Elisabeth 3010 Weller-Heinemann, Frank 3010 Windschall, Daniel 3010 Wittkowski, Helmut 4000 Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis$ddata from the German AID-registry [Lainka, Elke] 4209 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. 4950 https://doi.org/10.1186/s12969-021-00510-8$xR$3Volltext$534 4950 https://nbn-resolving.org/urn:nbn:de:hbz:465-20240904-091407-2$xR$3Volltext$534 4961 https://duepublico2.uni-due.de/receive/duepublico_mods_00077545 5051 610 5550 Anakinra 5550 Autoinflammatory disease 5550 Canakinumab 5550 Interleukin-1 5550 Proinflammatory cytokines 5550 Systemic juvenile idiopathic arthritis