Vasculitic neuropathy results from inflammation within the blood vessel walls of the vasa nervorum, the blood vessels that supply peripheral nerves. The forms of systemic vasculitis most likely to be associated with vasculitic neuropathy are polyarteritis nodosa, microscopic polyangiitis, the Churg—Strauss syndrome, Wegener's granulomatosis, and mixed cryoglobulinemia. Large-vessel vasculitides such as giant cell arteritis and Takayasu's arteritis seldom cause vasculitic neuropathy. Similarly, vasculitic neuropathy is seldom (if ever) a complication of the types of vasculitis that involve the smallest blood vessels, e.g., cutaneous leu-kocytoclastic angiitis (hypersensitivity vasculitis) and Henoch—Schönlein purpura. Rather, vasculitic neuropathy tends to be caused by systemic vasculitides that have the ability to target medium-sized blood vessels. Vasculitic neuropathy can also occur as a disease process that is limited to the peripheral nerves, sparing other organs. Such cases are sometimes referred to as “isolated vasculitis of the peripheral nervous system” or “nonsystemic vasculitic neuropathy.” Vasculitic neuropathy is generally a “length-dependent” disease process. Thus, the distal extremities tend to be involved more extensively than are the proximal extremities and trunk importantly, vasculitic neuropathy can also present initally as a multifocal, nonlength dependent process (arms affected earlier than legs). Vasculitic neuropathy has the potential to cause serious morbidity (persisting weakness) through the resultant infarctions of peripheral nerves. The combination of cyclophosphamide and high doses of glucocorticoids is an appropriate treatment regimen for most patients with vasculitic neuropathy.
KeywordsGiant Cell Arteritis Nerve Conduction Study Systemic Vasculitis Nerve Biopsy Mixed Cryoglobulinemia
- Schaumburg HH, Berger AR, Thomas PK. Disorders of peripheral nerves (Contemporary Neurology Series 36). 2nd ed. Philadelphia: FA Davis; 1992. p. 130–5Google Scholar
- Vrancken AF, Hughes RA, Said G, et al Immunosuppressive treatment for non-systemic vasculitic neuropathy. Cochrane Database Syst Rev. 2007;24(1):CD006050Google Scholar