PURPOSE OF REVIEW 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.

RECENT FINDINGS 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.

SUMMARY 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.

Address correspondence to Dr Chafic Karam, 3303 S Bond Ave, 18th Floor, Portland, OR 97239, chafickaram@gmail.com.

RELATIONSHIP DISCLOSURE: Dr Karam has served as a deputy editor for Neurology and as a consultant for Acceleron Pharma, Inc; Akcea Therapeutics; Alnylam Pharmaceuticals, Inc; Argenx; Biogen; CSL Behring; and Sanofi Genzyme. Dr Karam has received personal compensation for speaking engagements from Akcea Therapeutics; Alnylam Pharmaceuticals, Inc; CSL Behring; and Sanofi Genzyme and research/grant support from Akcea Therapeutics and Sanofi Genzyme.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Karam discusses the unlabeled/investigational use of cyclophosphamide, immunoglobulin, rituximab, and steroids in the treatment of vasculitis and immune-mediated neuropathy in rheumatologic disorders.