Vasculitic Neuropathies

  • Elie Naddaf
  • P. James Bonham Dyck
Neuromuscular Disorders (SA Rudnicki, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Neuromuscular Disorders

Opinion statement

From pathological standpoint, we divide vasculitic neuropathies in two categories: nerve large arteriole vasculitides and nerve microvasculitis. It is also important to determine whether a large arteriole vasculitis has an infectious etiology as it entails different treatment approach. Treatment of non-infectious large arteriole vasculitides consists initially of induction therapy with corticosteroids. Adding an immunosuppressant, mainly cyclophosphamide, is often needed. Treatment of infectious large arteriole vasculitides needs a multidisciplinary approach to target both the underlying infection and the vasculitis. Corticosteroids are the first-line therapy for classic non-systemic vasculitic neuropathy. Stable or improving patients without biopsy evidence of active vasculitis can be either observed or treated. Currently, adding an immunosuppressant is only indicated for patients who continue to progress on corticosteroids alone or patients with a rapidly progressive course. The treatment of the radiculoplexus neuropathies such as diabetic lumbosacral radiculoplexus neuropathy, lumbosacral radiculoplexus neuropathy (in non-diabetic patients), and diabetic cervical radiculoplexus neuropathy, as well as painless diabetic motor neuropathy, is not well established yet. We treat patients, if they present early on in the disease course or if they have severe disabling symptoms, with IV methylprednisolone 1 g once a week for 12 weeks.


Vasculitic neuropathy Necrotizing vasculitis Nerve large arteriole Microvasculitis Microscopic polyangiitis Polyarteritis nodosa Churg-Strauss syndrome Wegener’s granulomatosis Rheumatoid vasculitis Sjögren’s syndrome Hepatitis C vasculitic neuropathy Cryoglobulinemia HIV-associated vasculitic neuropathy Non-systemic vasculitic neuropathy Diabetic lumbosacral radiculoplexus neuropathy Lumbosacral radiculoplexus neuropathy Diabetic cervical radiculoplexus neuropathy Painless diabetic motor neuropathy 



We thank Janean Engelstad for her assistance with the figures.

Compliance with Ethics Guidelines

Conflict of Interest

Elie Naddaf and P. James Bonham Dyck declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  1. 1.Department of NeurologyMayo ClinicRochesterUSA

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