Diagnosis and treatment of vasculitic neuropathy
- Steven David Brass, MD, MPH, MBA
Steven David Brass, MD, MPH, MBA
- Director of Medical Affairs
- Providence Little Company of Mary Medical Center
- Simon M Helfgott, MD
Simon M Helfgott, MD
- Associate Professor of Medicine
- Harvard Medical School
- Section Editors
- Peter A Merkel, MD, MPH
Peter A Merkel, MD, MPH
- Section Editor — Vasculitis
- Chief, Division of Rheumatology
- University of Pennsylvania
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
Vasculitic diseases can affect any organ or tissue, including the peripheral nerves. When vasculitis affects the vasa nervorum, the results are often critical ischemia to peripheral nerves and potentially profound clinical sequelae.
Vasculitic neuropathy is usually only one feature of a systemic condition that can involve the skin, lungs, kidneys, and other organs. As an example, the group of vasculitic disorders commonly associated with antineutrophil cytoplasmic antibodies (ANCA) (ie, granulomatosis with polyangiitis [Wegener’s], microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis [Churg-Strauss]) often cause vasculitic neuropathy. Polyarteritis nodosa (PAN) and mixed cryoglobulinemia are also prone to involve the peripheral nervous system.
In a minority of patients with vasculitic neuropathy, the peripheral nervous system vasculitis occurs in isolation. In this setting, the disorder is referred to as nonsystemic vasculitic neuropathy or isolated peripheral nervous system vasculitis [1-4].
Even in the absence of frank systemic vasculitis, nonsystemic vasculitic neuropathy can be a devastating illness, leading to severe functional disabilities from which some patients never recover fully. Because the regeneration of peripheral nerves may require months or years, the goals of therapy are to control the inflammation within the vasa nervorum as quickly as possible to prevent or minimize nerve damage.
This topic will review the diagnosis and treatment of vasculitic peripheral neuropathy. The clinical manifestations of vasculitic neuropathy are presented separately. (See "Clinical manifestations of vasculitic neuropathy".)
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- DIAGNOSTIC EVALUATION
- Laboratory testing
- - ANA
- - Specific ANAs
- - ANCA
- - Cryoglobulins and RF
- Electrodiagnostic studies
- Nerve/muscle biopsy
- DIFFERENTIAL DIAGNOSIS
- TREATMENT OF NONSYSTEMIC DISEASE
- - Tapering regimen
- Pulse glucocorticoid therapy
- Maintenance therapy
- Duration of therapy
- SUMMARY AND RECOMMENDATIONS