Bielak, M., Husmann, E., Weyandt, N., Haas, J. -P., Huegle, B., Horneff, G., Neudorf, U., Lutz, T., Lilienthal, E., Kallinich, T., Tenbrock, K., Berendes, R., Niehues, T., Wittkowski, H., Weissbarth-Riedel, E., Heubner, G., Oommen, P., Klotsche, J., Foell, Dirk and Lainka, E. (2018). IL-6 blockade in systemic juvenile idiopathic arthritis - achievement of inactive disease and remission (data from the German AID-registry). Pediatr. Rheumatol., 16. LONDON: BIOMED CENTRAL LTD. ISSN 1546-0096

Full text not available from this repository.

Abstract

Background: Systemic juvenile idiopathic arthritis (sJIA) is a complex disease with an autoinflammatory component of unknown etiology related to the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines like interleukin-6 (IL-6), and effective drugs inhibiting their signaling are being developed. This study evaluates sJIA patients treated with the IL-6 inhibitor tocilizumab (TCZ) concerning clinical response rate, disease course and adverse effects in a real-life clinical setting. Methods: In 2009 a clinical and research consortium was established, including an online registry for autoinflammatory diseases (AID) (https://aid-register.de). Data for this retrospective TCZ study were documented by 13 centers. Results: From 7/2009 to 4/2014, 200 patients with sJIA were recorded in the AID-registry. Out of these, 46 (19 m, 27 f, age 1-18 years) received therapy with TCZ. Long term treatment (median 23 months) has been documented in 24/46 patients who were evaluated according to Wallace criteria (active disease 6/24, inactive disease 5/24, remission 13/24 cases). Under observation co-medication were used in 40/46 cases. Adverse events were reported in 11/46 patients. The clinical response rate (no clinical manifestation, no increased inflammation parameters) within the first 12 weeks of treatment was calculated to be 35%. Conclusion: Out of 200 sJIA children reported in the German AID-registry, 46 were treated with TCZ, showing a clinical response rate of 35% during the first 12 weeks, and inactive disease and/or remission under medication in 75% after one year. Adverse events were seen in 24% and severe adverse events in 4%.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Bielak, M.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Husmann, E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weyandt, N.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Haas, J. -P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Huegle, B.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Horneff, G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Neudorf, U.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lutz, T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lilienthal, E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kallinich, T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tenbrock, K.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Berendes, R.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Niehues, T.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wittkowski, H.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Weissbarth-Riedel, E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Heubner, G.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oommen, P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klotsche, J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Foell, DirkUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lainka, E.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-189642
DOI: 10.1186/s12969-018-0236-y
Journal or Publication Title: Pediatr. Rheumatol.
Volume: 16
Date: 2018
Publisher: BIOMED CENTRAL LTD
Place of Publication: LONDON
ISSN: 1546-0096
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
MACROPHAGE ACTIVATION SYNDROME; RANDOMIZED-TRIALS; TOCILIZUMAB; SAFETY; EFFICACY; CLASSIFICATION; CANAKINUMAB; MANAGEMENT; CHILDREN; FEATURESMultiple languages
Pediatrics; RheumatologyMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/18964

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item