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September 11, 2017 E-Pearl of the Week: Nonsystemic vasculitic neuropathy (NSVN)

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September 11, 2017

Nonsystemic vasculitic neuropathy (NSVN)

Blood vessels supplying peripheral nerves, known as the vasa nervorum, are subject to inflammation. This weakens the blood vessel wall, causing ischemia and ultimately painful neuropathy.1 If the vasculitic neuropathy is not part of a systemic process, confined to the peripheral nerves only, it is called nonsystemic vasculitic neuropathy (NSVN). It is a painful motor neuropathy, presenting with subacute pain, weakness, and/ or numbness, and is often multifocal, asymmetric and distal-predominant. The ulnar and peroneal nerves, in particular, are commonly affected.2 Diagnostic criteria include EMG and nerve biopsy. Pathology varies based on the stage of the vasculitis, but commonly reveals T-cell predominant infiltrates with transmural inflammation, destruction of the vascular wall, and fibrinoid necrosis. NSVN is treated with immunosuppressive therapy, usually corcticosteroids. If rapidly progressive, an additional agent, such as cyclophosphamide, is added. 3 Clinical monitoring for other organ involvement over time is also important, as NSVN may develop into a systemic vasculitic neuropathy.

References:

  1. Lacomis D, Zivković SA. Approach to vasculitic neuropathies. J Clin Neuromuscul Dis 2007; 9: 265-276.
  2. Gorson KC. Vasculitic neuropathies: an update. Neurologist 2007; 13: 12-19.
  3. Collins MP, Dyck PJ, Gronseth GS et al. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst 2010; 15: 176-184.

Submitted by Danielle Stember, MD - Neurology Resident, NYU School of Medicine.

Dr. Stember reports no disclosures.

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