Lainka, Elke, Baehr, Melanie, Raszka, Bernadette, Haas, Johannes-Peter, Huegle, Boris, Fischer, Nadine, Foell, Dirk, Hinze, Claas, Weissbarth-Riedel, Elisabeth, Kallinich, Tilmann, Horneff, Gerd, Windschall, Daniel, Lilienthal, Eggert, Niehues, Tim, Neudorf, Ulrich, Berendes, Rainer, Kuester, Rolf-Michael, Oommen, Prasad Thomas, Rietschel, Christoph, Lutz, Thomas, Weller-Heinemann, Frank, Tenbrock, Klaus, Heubner, Georg Leonhard, Klotsche, Jens and Wittkowski, Helmut (2021). Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis - data from the German AID-registry. Pediatr. Rheumatol., 19 (1). LONDON: BMC. ISSN 1546-0096

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Abstract

BackgroundSystemic 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-1 beta (IL-1 beta), 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).MethodsIn 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.ResultsIn 6years, 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 12months 75/111 (ANA 55, CANA 20) patients were evaluated according to Wallace criteria (achievement of inactive disease 28/55 and 17/20, remission over 6months 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.ConclusionIn 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.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Lainka, ElkeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Baehr, MelanieUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Raszka, BernadetteUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haas, Johannes-PeterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huegle, BorisUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fischer, NadineUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Foell, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Hinze, ClaasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weissbarth-Riedel, ElisabethUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kallinich, TilmannUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Horneff, GerdUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Windschall, DanielUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lilienthal, EggertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niehues, TimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neudorf, UlrichUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berendes, RainerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kuester, Rolf-MichaelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oommen, Prasad ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rietschel, ChristophUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lutz, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weller-Heinemann, FrankUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tenbrock, KlausUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heubner, Georg LeonhardUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klotsche, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wittkowski, HelmutUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-604411
DOI: 10.1186/s12969-021-00510-8
Journal or Publication Title: Pediatr. Rheumatol.
Volume: 19
Number: 1
Date: 2021
Publisher: BMC
Place of Publication: LONDON
ISSN: 1546-0096
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
INTERLEUKIN-1 RECEPTOR ANTAGONIST; PRELIMINARY CRITERIA; CLASSIFICATION; REMISSION; EFFICACY; DISEASE; MULTICENTER; TOCILIZUMAB; CANAKINUMAB; SAFETYMultiple languages
Pediatrics; RheumatologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/60441

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