Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry
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 m...
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| Format: | Article |
| Language: | English |
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BMC
2021-03-01
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| Series: | Pediatric Rheumatology Online Journal |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12969-021-00510-8 |
| _version_ | 1818574212018733056 |
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| author | Elke Lainka Melanie Baehr Bernadette Raszka Johannes-Peter Haas Boris Hügle Nadine Fischer Dirk Foell Claas Hinze Elisabeth Weissbarth-Riedel Tilmann Kallinich Gerd Horneff Daniel Windschall Eggert Lilienthal Tim Niehues Ulrich Neudorf Rainer Berendes Rolf-Michael Küster Prasad Thomas Oommen Christoph Rietschel Thomas Lutz Frank Weller-Heinemann Klaus Tenbrock Georg Leonhard Heubner Jens Klotsche Helmut Wittkowski |
| author_facet | Elke Lainka Melanie Baehr Bernadette Raszka Johannes-Peter Haas Boris Hügle Nadine Fischer Dirk Foell Claas Hinze Elisabeth Weissbarth-Riedel Tilmann Kallinich Gerd Horneff Daniel Windschall Eggert Lilienthal Tim Niehues Ulrich Neudorf Rainer Berendes Rolf-Michael Küster Prasad Thomas Oommen Christoph Rietschel Thomas Lutz Frank Weller-Heinemann Klaus Tenbrock Georg Leonhard Heubner Jens Klotsche Helmut Wittkowski |
| author_sort | Elke Lainka |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-12-15T00:23:34Z |
| format | Article |
| id | doaj.art-1a94204ced604d9eb6be9bc5b08b8bfb |
| institution | Directory Open Access Journal |
| issn | 1546-0096 |
| language | English |
| last_indexed | 2024-12-15T00:23:34Z |
| publishDate | 2021-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Pediatric Rheumatology Online Journal |
| spelling | doaj.art-1a94204ced604d9eb6be9bc5b08b8bfb2022-12-21T22:42:13ZengBMCPediatric Rheumatology Online Journal1546-00962021-03-0119111210.1186/s12969-021-00510-8Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registryElke Lainka0Melanie Baehr1Bernadette Raszka2Johannes-Peter Haas3Boris Hügle4Nadine Fischer5Dirk Foell6Claas Hinze7Elisabeth Weissbarth-Riedel8Tilmann Kallinich9Gerd Horneff10Daniel Windschall11Eggert Lilienthal12Tim Niehues13Ulrich Neudorf14Rainer Berendes15Rolf-Michael Küster16Prasad Thomas Oommen17Christoph Rietschel18Thomas Lutz19Frank Weller-Heinemann20Klaus Tenbrock21Georg Leonhard Heubner22Jens Klotsche23Helmut Wittkowski24Department of Pediatric Rheumatology, University Children’s Hospital EssenDepartment of Pediatric Rheumatology, University Children’s Hospital EssenDepartment of Pediatric Rheumatology, University Children’s Hospital EssenGerman Center for Pediatric and Adolescent RheumatologyGerman Center for Pediatric and Adolescent RheumatologyGerman Center for Pediatric and Adolescent RheumatologyDepartment of Pediatric Rheumatology and Immunology, University of MuensterDepartment of Pediatric Rheumatology and Immunology, University of MuensterPediatric Rheumatology, University Children’s Hospital Hamburg-EppendorfDepartment of Pediatric Pneumology, Immunology and Intensive Medicine and Center for Chronically Sick Children, Charité University Medicine Berlin and German Rheumatism Research Centre BerlinDepartment of Pediatrics, Asklepios Clinic, Centre for Pediatric Rheumatology, St. Augustin and Medical Faculty, University of CologneDepartment of Pediatric Rheumatology, St. Josef HospitalDepartment of Pediatrics, Ruhr-University BochumHELIOS Children’s Hospital, Pediatric Immunology and RheumatologyDepartment of Pediatric Rheumatology, University Children’s Hospital EssenDepartment of Pediatric Rheumatology, St. Marien’s Children’s Hospital LandshutOrthopedics centre Altona and Pediatric practice RissenDepartment of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health, Medical Faculty, Heinrich-Heine-University DuesseldorfDepartment of Pediatrics, Clementine Children’s Hospital FrankfurtCenter for Pediatric and Adolescent Medicine/Pediatric Rheumatology, University Hospital HeidelbergDivision of Pediatric Rheumatology, Prof. Hess Children’s HospitalDepartment of Pediatric Pneumology, Allergology and Immunology, RWTH AachenDepartment of Pediatrics, Municipal Hospital DresdenGerman Rheumatism Research Centre BerlinDepartment of Pediatric Rheumatology and Immunology, University of MuensterAbstract 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.https://doi.org/10.1186/s12969-021-00510-8Systemic juvenile idiopathic arthritisAutoinflammatory diseaseProinflammatory cytokinesInterleukin-1AnakinraCanakinumab |
| spellingShingle | Elke Lainka Melanie Baehr Bernadette Raszka Johannes-Peter Haas Boris Hügle Nadine Fischer Dirk Foell Claas Hinze Elisabeth Weissbarth-Riedel Tilmann Kallinich Gerd Horneff Daniel Windschall Eggert Lilienthal Tim Niehues Ulrich Neudorf Rainer Berendes Rolf-Michael Küster Prasad Thomas Oommen Christoph Rietschel Thomas Lutz Frank Weller-Heinemann Klaus Tenbrock Georg Leonhard Heubner Jens Klotsche Helmut Wittkowski Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry Pediatric Rheumatology Online Journal Systemic juvenile idiopathic arthritis Autoinflammatory disease Proinflammatory cytokines Interleukin-1 Anakinra Canakinumab |
| title | Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry |
| title_full | Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry |
| title_fullStr | Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry |
| title_full_unstemmed | Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry |
| title_short | Experiences with IL-1 blockade in systemic juvenile idiopathic arthritis – data from the German AID-registry |
| title_sort | experiences with il 1 blockade in systemic juvenile idiopathic arthritis data from the german aid registry |
| topic | Systemic juvenile idiopathic arthritis Autoinflammatory disease Proinflammatory cytokines Interleukin-1 Anakinra Canakinumab |
| url | https://doi.org/10.1186/s12969-021-00510-8 |
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