Skip to content

Advertisement

  • Poster presentation
  • Open Access

Interleukin (IL)- 6 inhibition - Follow-up data of the German AID-registry1

  • 1,
  • 1,
  • 1,
  • 2,
  • 3,
  • 4,
  • 5,
  • 6,
  • 7,
  • 8,
  • 9,
  • 10,
  • 11,
  • 12,
  • 13,
  • 14,
  • 1,
  • 10,
  • 9 and
  • 1
Pediatric Rheumatology201513 (Suppl 1) :P63

https://doi.org/10.1186/1546-0096-13-S1-P63

  • Published:

Keywords

  • Lymphoma
  • Proinflammatory Cytokine
  • Innate Immune System
  • Leukopenia
  • Unknown Etiology

Introduction

Systemic juvenile idiopathic arthritis (SJIA) is regarded as an autoinflammatory disease (AID) of unknown etiology related to abnormalities of the innate immune system. A major role in the pathogenesis has been ascribed to proinflammatory cytokines as interleukin (IL)-6 and IL-1.

Objectives

Analysis of treatment results with the IL-6 inhibitor tocilizumab

Patients and methods

From 7/2009 to 4/2014 200 patients with SJIA were documented in the AID-registry. 46 of 200 patients (19 m, 27 f) at the age of 1-18 years (median 9) received therapy with tocilizumab (median 13 months, range 1-48). 24 of 46 patients received long term treatment (median 23 months, range 12-48) and were evaluated concerning Wallace criteria [1]. Different clinical courses were continuous (C) n=12, polycyclic (PC) n=16, arthritic (A) n=18. Besides we estimated a response rate (definition: no clinical manifestation and no inflammation parameters) in the first 12 weeks of treatment. Data are based on the AID-Registry (http://www.aid-register.de).

Results

According to Kaplan-Meier analysis 30% reached inactive disease or remission after the first 12 weeks of treatment. A rapid response to tocilizumab seems to be related to long term inactivity of SJIA. Comparison of the three disease courses (PC, C, A) revealed significant differences in the outcome; polycyclic courses show the fastest response followed by continuous courses. Worst outcome was evaluated in arthritic courses. Wallace criteria measured after at least 12 months: remission 54%, active disease 25%, inactive disease 21%. 4 (9%) patients were non-responders over the whole time. 60% of the patients showed no measurable CRP within the first 4 weeks and during tocilizumab therapy. Adverse events were reported in 11 (24%) patients: most leukopenia, infections and elevated transaminases, one Hodgkin's lymphoma, one gut perforation.

Conclusion

A significant proportion of patients documented with SJIA in der German AID-registry is treated with tocilizumab (23%). We estimated a good response in the first 12 weeks of therapy of 30% and also by Wallace of 76% (inactive disease or remission). The response appears to depend on different disease phenotypes.

1The AID-Registry is funded by the BMBF (01GM08104, 01GM1112D)

Authors’ Affiliations

(1)
Universitätsklinikum Essen, Kinderklinik, Essen, Germany
(2)
Klinik, Kinder- und Jugendrheumatologie, Garmisch-Partenkirchen, Germany
(3)
Asklepios Klinik St. Augustin, Pädiatrie, St. Augustin, Germany
(4)
Universitätsklinikum, Pädiatrie, Heidelberg, Germany
(5)
Ruhr-Universität Bochum, Pädiatrie, Bochum, Germany
(6)
Universitätsklinikum, Pädiatrie, Berlin, Germany
(7)
Universitätsklinikum, Pädiatrie, Aachen, Germany
(8)
Klinik, Pädiatrie, Landshut, Germany
(9)
Helios Klinik, Pädiatrie, Krefeld, Germany
(10)
Universitätsklinikum, Pädiatrische Rheumatologie, Münster, Germany
(11)
Universitätsklinikum, Pädaitrische Rheumatologie, Hamburg, Germany
(12)
Städtisches Krankenhaus, Pädiatrie, Dresden, Germany
(13)
Universitätsklinikum, Pädiatrie, Düsseldorf, Germany
(14)
DRFZ, Epidemiologie, Berlin, Germany

References

  1. Wallace CA, et al: J Rheumatol. 2004, 31: 2290-4.PubMedGoogle Scholar

Copyright

Advertisement