Elke Lainka1, Melanie Baehr2, Bernadette Raszka2, Johannes-Peter Haas3, Boris Hügle3, Nadine Fischer3, Dirk Foell4, Claas Hinze4, Elisabeth Weissbarth-Riedel5, Tilmann Kallinich6, Gerd Horneff7, Daniel Windschall8, Eggert Lilienthal9, Tim Niehues10, Ulrich Neudorf2, Rainer Berendes11, Rolf-Michael Küster12, Prasad Thomas Oommen13, Christoph Rietschel14, Thomas Lutz15, Frank Weller-Heinemann16, Klaus Tenbrock17, Georg Leonhard Heubner18, Jens Klotsche19, Helmut Wittkowski4. 1. Department of Pediatric Rheumatology, University Children's Hospital Essen, Essen, Germany. elke.lainka@uni-due.de. 2. Department of Pediatric Rheumatology, University Children's Hospital Essen, Essen, Germany. 3. German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany. 4. Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany. 5. Pediatric Rheumatology, University Children's Hospital Hamburg-Eppendorf, Hamburg, Germany. 6. Department of Pediatric Pneumology, Immunology and Intensive Medicine and Center for Chronically Sick Children, Charité University Medicine Berlin and German Rheumatism Research Centre Berlin, Berlin, Germany. 7. Department of Pediatrics, Asklepios Clinic, Centre for Pediatric Rheumatology, St. Augustin and Medical Faculty, University of Cologne, Cologne, Germany. 8. Department of Pediatric Rheumatology, St. Josef Hospital, Sendenhorst, Germany. 9. Department of Pediatrics, Ruhr-University Bochum, Bochum, Germany. 10. HELIOS Children's Hospital, Pediatric Immunology and Rheumatology, Krefeld, Germany. 11. Department of Pediatric Rheumatology, St. Marien's Children's Hospital Landshut, Landshut, Germany. 12. Orthopedics centre Altona and Pediatric practice Rissen, Hamburg, Germany. 13. Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany. 14. Department of Pediatrics, Clementine Children's Hospital Frankfurt, Frankfurt, Germany. 15. Center for Pediatric and Adolescent Medicine/Pediatric Rheumatology, University Hospital Heidelberg, Heidelberg, Germany. 16. Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Germany. 17. Department of Pediatric Pneumology, Allergology and Immunology, RWTH Aachen, Aachen, Germany. 18. Department of Pediatrics, Municipal Hospital Dresden, Dresden, Germany. 19. German Rheumatism Research Centre Berlin, Berlin, Germany.
Abstract
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.
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.
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