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Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature

  • Martin KruscheEmail author
  • Nikolas Ruffer
  • Ina Kötter
Case Based Review

Abstract

Polyarteritis nodosa (PAN) is a rare systemic vasculitis affecting multiple organs. Current standard treatment includes the use of glucocorticoids and cyclophosphamide. Unfortunately, some patients do not respond to this treatment and other therapeutic options are needed. We present a case of a young male with refractory PAN and ongoing biopsy evidence of active vasculitis despite optimal standard therapies, who was successfully treated with interleukin-6 antagonist, tocilizumab. A 24-year-old male presented with severe immobilizing polyneuropathy and myalgias. Clinical features included fasciitis, tenosynovitis, early signs of polyneuropathy, and panniculitis, which were largely refractory to the standard therapies. The previous unsuccessful treatments included high-dose glucocorticoids, methotrexate, cyclophosphamide, rituximab, anakinra, and intravenous immunoglobulins. Magnetic resonance imaging showed signs of myositis, with muscle biopsy confirming the diagnosis of PAN. Rapid clinical improvement and sustained remission occurred after interleukin-6 inhibition with tocilizumab at increased dose of 800 mg every 4 weeks. The used search strategy identified 20 publications of which four articles were included for the further analysis. In total, we report the clinical outcome of five PAN cases from the literature and the present one. The present case and the systematic review of literature suggest that tocilizumab is a possible treatment option for, otherwise, refractory hepatitis B virus negative PAN. Randomized-controlled trials are required to evaluate the safety and efficacy of tocilizumab in PAN.

Keywords

Polyarteritis nodosa Tocilizumab Biological therapy Myositis Vasculitis 

Notes

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Compliance with ethical standards

Conflict of interest

Martin Krusche reports receiving financial support from Chugai for an educational program and honorarium for a talk. Nikolas Ruffer declares that he has no conflict of interest. Ina Kötter reports receiving honorarium from Chugai and Roche for talks.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Declaration of Helsinki and its later amendments or comparable ethical standards. All authors fulfilled the ICMJE authorship criteria.

Informed consent

Informed consent was obtained from the individual participant included in the study.

Supplementary material

296_2018_4210_MOESM1_ESM.docx (50 kb)
Supplementary material 1 (DOCX 50 KB)

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Asklepios Klinik Altona, Rheumatologie, Klinische Immunologie, NephrologieHamburgGermany

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