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  • Review Article
  • Published:

The nonsystemic vasculitic neuropathies

Nature Reviews Neurology volume 13, pages 302–316 (2017)Cite this article

Subjects

Key Points

  • Nonsystemic vasculitic neuropathy (NSVN) is typically multifocal or asymmetric, painful, sensory or sensorimotor, lower-limb predominant, and characterized by one or more acute attacks

  • NSVN is usually distal-predominant, but can involve proximal nerves, a phenotype designated radiculoplexus neuropathy (diabetic or nondiabetic)

  • The diagnostic gold standard for NSVN is vessel wall inflammation and damage identified by nerve biopsy; a probable diagnosis is possible if the biopsy findings are suspicious but not pathognomonic

  • We propose a clinical definition and differential diagnosis of a multifocal pattern of neuropathy ('mononeuritis multiplex')

  • NSVN and neuropathy-predominant systemic vasculitis should probably be treated with an immunosuppressive agent in addition to corticosteroids.

Abstract

Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and — arguably — neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin–nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard definitions, resulting in inconsistencies between studies. We propose definitions and provide an up-to-date differential diagnosis of multifocal neuropathy. Available evidence suggests that NSVN and neuropathy-predominant systemic vasculitis might be controlled better by treatment with corticosteroids and an immunosuppressive agent than with corticosteroids alone. Treated NSVN rarely spreads to other organs, but 30% of patients experience a relapse. Long-term neurological outcome is favourable, but chronic pain is common.

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Figure 1: Anatomical patterns of neuropathy.
Figure 2: Algorithm for immunosuppressive treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides.

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Authors and Affiliations

  1. Department of Neurology, Medical College of Wisconsin, 9200 W Wisconsin Ave., Milwaukee, Wisconsin 53226, Wisconsin, USA

    Michael P. Collins

  2. Department of Neurology, King's College Hospital, London, SE5 9RS, Denmark Hill, UK

    Robert D. Hadden

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  1. Michael P. Collins
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  2. Robert D. Hadden
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Both authors researched the data for the article, provided substantial contributions to discussions of its content, wrote the article and undertook review and/or editing of the manuscript before submission.

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Correspondence to Michael P. Collins.

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Supplementary information

Supplementary information S1 (table)

Prevalence of vasculitis types in unselected series of vasculitic neuropathy (PDF 172 kb)

Supplementary information S2 (table)

Clinicopathologic characteristics of the ten largest cohorts of nonsystemic vasculitic neuropathy (PDF 162 kb)

Supplementary information S3 (table)

New evidence on histopathological factors associated with vasculitic neuropathy (PDF 121 kb)

Supplementary information S4 (table)

Evidence on clinical and laboratory factors associated with vasculitic neuropathy (PDF 163 kb)

Supplementary information S5 (box)

Differential diagnosis of multifocal/asymmetric neuropathy (PDF 148 kb)

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Collins, M., Hadden, R. The nonsystemic vasculitic neuropathies. Nat Rev Neurol 13, 302–316 (2017). https://doi.org/10.1038/nrneurol.2017.42

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