Vasculitic Neuropathies

Abstract

Vasculitic neuropathies are disorders that result from inflammation in the peripheral nerves' vascular supply, resulting in ischemic injury. These disorders may be a result of systemic inflammation or may be confined to the peripheral nervous system. Causative etiologies include primary systemic vasculitis, vasculitis secondary to other conditions such as primary connective tissue disorders, infectious, paraneoplastic, and drug-induced conditions, and nonsystemic vasculitic neuropathy. Early recognition and treatment of these conditions is imperative to prevent substantial morbidity and mortality. The goal of this review is to provide an organization of the vasculitic neuropathies and an overview of principles of diagnosis and treatment for the clinical neurologist.

Peripheral neuropathy in systemic vasculitis and other autoimmune diseases – a report of five cases emphasizing the importance of etiologic characterization

“…Vasculitic neuropathy has a characteristic clinical presentation with progressive sensorimotor symptoms developing over weeks to months [ 5 , 7 ]. Most patients experience one or more acute attacks, with a stepwise installation of the deficits, but one-third have a chronic slowly progressive course [ 5 , 7 , 8 ]. Peripheral nervous system vasculitis exhibits three patterns of clinical involvement [ 3 , 7 ].…”

“…The commonest and most distinctive one is a multiple mononeuropathy, occurring in 35–65% of the patients with vasculitic neuropathy [ 3 , 5 ]. However, these patients may also present with a distal asymmetric polyneuropathy or, more rarely, a distal symmetric polyneuropathy [ 3 , 8 ]. In this series, all the patients presented with an asymmetric sensorimotor deficit, with a stepwise progression, featuring a pattern of overlapping multiple mononeuropathies, evolving to distal asymmetric polyneuropathies in the first four patients.…”

“…Either primary or secondary to autoimmune diseases, vasculitis affecting small arteries or large arterioles are more likely to produce a peripheral neuropathy [ 3 , 4 ]. The primary vasculitis most commonly associated with neuropathy includes polyarteritis nodosa (PAN), essential mixed cryoglobulinemic and anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis [ [5] , [6] , [7] , [8] ]. Among the autoimmune diseases, rheumatoid arthritis, hepatitis B virus (HBV)-associated PAN, and hepatitis C virus (HCV)-related cryoglobulinemic vasculitis are the most frequent [ 5 , 7 , 8 ].…”

Role of Intravenous Lidocaine Infusion in the Treatment of Peripheral Neuropathy

“…Severe systemic vasculitis is treated with corticosteroids initially, and cyclophosphamide or rituximab can be added if symptoms persist, while non-systemic vasculitis is treated with corticosteroids only. 31…”

Central and Peripheral Nervous System Complications of Vasculitis Syndromes From Pathology to Bedside: Part 1—Central Nervous System

“…The typical CNS manifestation of vasculitis is a stroke, either because of SOV or neurological consequence of a systemic vasculitis. Other common CNS presentations include visual loss, encephalopathy, seizures, headache, venous thrombosis, and hemorrhagic stroke [ 2 ••], whereas the involvement of PNS usually presents as peripheral neuropathy of different types (polyneuropathy, mononeuropathy, or multiplex mononeuropathy, and cranial neuropathy) and neuromuscular disease [ 3 , 4 ]. Overall, differential diagnosis of vasculitis is challenging, and neurologists are often on the frontline of the diagnosis and treatment of these patients.…”