Universität zu Köln

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

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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:	Creators Email ORCID ORCID Put Code Bielak, M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Husmann, E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weyandt, N. UNSPECIFIED UNSPECIFIED UNSPECIFIED Haas, J. -P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Huegle, B. UNSPECIFIED UNSPECIFIED UNSPECIFIED Horneff, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Neudorf, U. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lutz, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Lilienthal, E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kallinich, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Tenbrock, K. UNSPECIFIED UNSPECIFIED UNSPECIFIED Berendes, R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Niehues, T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wittkowski, H. UNSPECIFIED UNSPECIFIED UNSPECIFIED Weissbarth-Riedel, E. UNSPECIFIED UNSPECIFIED UNSPECIFIED Heubner, G. UNSPECIFIED UNSPECIFIED UNSPECIFIED Oommen, P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Klotsche, J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Foell, Dirk UNSPECIFIED UNSPECIFIED UNSPECIFIED Lainka, E. UNSPECIFIED UNSPECIFIED UNSPECIFIED
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:	Keywords Language MACROPHAGE ACTIVATION SYNDROME; RANDOMIZED-TRIALS; TOCILIZUMAB; SAFETY; EFFICACY; CLASSIFICATION; CANAKINUMAB; MANAGEMENT; CHILDREN; FEATURES Multiple languages Pediatrics; Rheumatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/18964