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Neuropediatrics 2019; 50(03): 193-196
DOI: 10.1055/s-0039-1684004
Short Communication
Georg Thieme Verlag KG Stuttgart · New York

Two Cases of Pediatric AQP4-Antibody Positive Neuromyelitis Optica Spectrum Disorder Successfully Treated with Tocilizumab

Markus Breu
1   Department of Pediatric and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Sarah Glatter
1   Department of Pediatric and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Romana Höftberger
2   Institute of Neurology, Medical University of Vienna, Vienna, Austria
,
Michael Freilinger
1   Department of Pediatric and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Karl Kircher
3   Department of Ophthalmology and Optometrics, Medical University of Vienna, Vienna, Austria
,
Gregor Kasprian
4   Department of Biomedical Imaging and Image-guided Therapy, Medical University of Vienna, Vienna, Austria
,
Rainer Seidl
1   Department of Pediatric and Adolescent Medicine, Medical University of Vienna, Vienna, Austria
,
Barbara Kornek
5   Department of Neurology, Medical University of Vienna, Vienna, Austria
› Author Affiliations Study Funding This study was supported in part by the grant “Jubiläumsfonds der Österreichischen Nationalbank, Project 16919” to R.H.
Further Information

Publication History

11 September 2018

04 February 2019

Publication Date:
26 March 2019 (online)

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Abstract

B cell depletion with the anti-CD20-antibody rituximab is widely considered treatment of choice for long-term immunotherapy in aquaporin-4 (AQP4)-antibody positive neuromyelitis optica spectrum disorder (NMOSD). However, up to 30% of patients suffer from relapses despite complete B cell depletion. In these cases, the IL6 (interleukin-6)-receptor blocking antibody tocilizumab has been suggested as an alternative. We report two female adolescents with AQP4-antibody positive NMOSD who relapsed under rituximab treatment and clinically stabilized after switching to monthly administrations of tocilizumab. Our data suggest that early escalation of therapy with tocilizumab may lead to stabilization of disease activity in pediatric NMOSD patients who relapse under B cell depletion.

Keywords

neuromyelitis optica - tocilizumab - pediatric - AQP4 - interleukin-6 - plasmablasts

Patient Consent

Written informed consent for patient information and MR images to be published was provided by the patients. The study was approved by the institutional review board (Ethikkomission der Medizinischen Universität Wien, No. EK1123_2015).


 

  • References

  • 1 Jacob A, Weinshenker BG, Violich I. , et al. Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol 2008; 65 (11) 1443-1448
    CrossrefPubMedGoogle Scholar
  • 2 Pellkofer HL, Krumbholz M, Berthele A. , et al. Long-term follow-up of patients with neuromyelitis optica after repeated therapy with rituximab. Neurology 2011; 76 (15) 1310-1315
    CrossrefPubMedGoogle Scholar
  • 3 Kim S-H, Kim W, Li XF, Jung IJ, Kim HJ. Repeated treatment with rituximab based on the assessment of peripheral circulating memory B cells in patients with relapsing neuromyelitis optica over 2 years. Arch Neurol 2011; 68 (11) 1412-1420
    CrossrefPubMedGoogle Scholar
  • 4 Araki M, Aranami T, Matsuoka T, Nakamura M, Miyake S, Yamamura T. Clinical improvement in a patient with neuromyelitis optica following therapy with the anti-IL-6 receptor monoclonal antibody tocilizumab. Mod Rheumatol 2013; 23 (04) 827-831
    CrossrefPubMedGoogle Scholar
  • 5 Chihara N, Aranami T, Sato W. , et al. Interleukin 6 signaling promotes anti-aquaporin 4 autoantibody production from plasmablasts in neuromyelitis optica. Proc Natl Acad Sci U S A 2011; 108 (09) 3701-3706
    CrossrefPubMedGoogle Scholar
  • 6 Hinson SR, Lennon VA, Pittock SJ. Autoimmune AQP4 channelopathies and neuromyelitis optica spectrum disorders. Handb Clin Neurol 2016; 133: 377-403
    PubMedGoogle Scholar
  • 7 Mealy MA, Wingerchuk DM, Palace J, Greenberg BM, Levy M. Comparison of relapse and treatment failure rates among patients with neuromyelitis optica: multicenter study of treatment efficacy. JAMA Neurol 2014; 71 (03) 324-330
    CrossrefPubMedGoogle Scholar
  • 8 Marino A, Narula S, Lerman MA. First pediatric patient with neuromyelitis optica and Sjögren syndrome successfully treated with tocilizumab. Pediatr Neurol 2017; 73: e5-e6
    CrossrefPubMedGoogle Scholar
  • 9 Ayzenberg I, Kleiter I, Schröder A. , et al. Interleukin 6 receptor blockade in patients with neuromyelitis optica nonresponsive to anti-CD20 therapy. JAMA Neurol 2013; 70 (03) 394-397
    CrossrefPubMedGoogle Scholar