This article discusses peripheral neuropathies associated with vasculitis (isolated or in the setting of systemic vasculitis) and autoimmune connective tissue disease and provides a brief overview of their diagnostic evaluation and management.
The classification of systemic vasculitic neuropathy and nonsystemic vasculitic neuropathy continues to evolve. Classification according to the presence of antineutrophil cytoplasmic antibodies and their subtypes facilitates prognostication and management. Recent research on antineutrophil cytoplasmic antibody–associated vasculitis has added to our understanding of its neurologic complications. The treatment of vasculitis is also evolving, and new nonsystemic vasculitic neuropathy classification has impacted the treatment and management of this disorder. New classification criteria for Sjögren syndrome (which commonly causes neurologic complications) facilitate accurate and timely diagnosis.
Vasculitis and autoimmune connective tissue disease are underrecognized and treatable causes of peripheral neuropathy. Furthermore, peripheral neuropathy may reveal an underlying rheumatologic or vasculitic disorder. Rapid recognition and treatment are essential. Familiarity with the diagnosis and treatment of neuropathies in the setting of connective tissue disease and vasculitis reduces morbidity and, in some cases, mortality.