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Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients

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Abstract

The objective of this study is to report the clinical presentation and long-term outcome of patients with non-systemic vasculitic neuropathy (NSVN) seen at our neuromuscular center. In this retrospective analysis, we assessed medical records of 60 patients with biopsy-proven NSVN (39 men, 21 women; median age: 64 years, 24–80), who were seen at our department between 1999 and 2008 and were followed up until 2014. The initial neurological findings, laboratory and neurophysiological data, treatment regimens, and outcome were analyzed in all patients. NSVN was mostly asymmetric (48/60, 80 %), sensorimotor (45/60, 75 %), and painful (38/60, 63 %), with walking impairment as one major sign (51/60, 85 %). No compound action potentials could be recorded in 29/60 (48 %) sural nerves (later biopsied side) and in 6/60 (10 %) tibial (motor) nerves. Pathology of sural nerve was informative in all cases irrespective of neurophysiological findings and prior immunosuppression. After initial treatment with i.v. methylprednisolone, all patients reported overall improvement. Of the 46 patients who were followed for >1 year, those with mild to moderate affliction were stable with azathioprine (19/46, 41 %), while 18/46 (39 %) patients were treated with cyclophosphamide and other immunosuppressants due to progression or relapse. At 4 years, 24/46 (52 %) patients had either discontinued (n = 21) or had primarily refused immunosuppressive treatment (n = 3) without relapse. Age younger than the group median of 64 years was associated with better outcome. No patient evolved to systemic vasculitis. NSVN is a potentially treatable disorder of the peripheral nervous system.

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Acknowledgements

Expert technical help by H. Klüpfel is gratefully acknowledged. The study was supported by intramural research funds of the University of Würzburg. N. Üçeyler and C. Sommer are funded by the European Union’s Seventh Framework Programme (“ncRNAPain”, grant agreement number 602133). KVT is a senior professor of neurology funded by the Medical School Research Fund.

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standards

We received approval from the Würzburg Medical Faculty Ethics Committee to utilize our patients’ clinical data and bio-samples for this research. All patients had given written consent for their bio-samples and data to be used.

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Authors and Affiliations

  1. Department of Neurology, University of Würzburg, Josef-Schneider-Str. 11, 97080, Würzburg, Germany

    Nurcan Üçeyler, Anna Geng, Karlheinz Reiners, Klaus V. Toyka & Claudia Sommer

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  1. Nurcan Üçeyler
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  2. Anna Geng
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  3. Karlheinz Reiners
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  4. Klaus V. Toyka
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  5. Claudia Sommer
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Correspondence to Nurcan Üçeyler.

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Üçeyler, N., Geng, A., Reiners, K. et al. Non-systemic vasculitic neuropathy: single-center follow-up of 60 patients. J Neurol 262, 2092–2100 (2015). https://doi.org/10.1007/s00415-015-7813-5

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  • DOI: https://doi.org/10.1007/s00415-015-7813-5

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