Distinctive clinical features in biopsy-proven nerve large-arteriole vasculitis and microvasculitis
Pannathat Soontrapa1,2, Marcus V Pinto1, Kamal Shouman1
1Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Brain : a Journal of Neurology
|December 23, 2024
Summary
Nerve large-arteriole vasculitis typically has an acute onset and distal polyneuropathy, often linked to autoimmune diseases. Nerve microvasculitis presents more chronically, affecting proximal nerves and autonomic function, frequently as non-systemic vasculitis.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Vasculitic neuropathy results from inflammatory destruction of nerve blood vessels, leading to ischemia.
- Nerve vasculitis is classified by vessel size: large-arteriole (≥75 µm) and microvasculitis (<75 µm).
Purpose of the Study:
- To characterize and compare the clinical features of nerve large-arteriole vasculitis and nerve microvasculitis.
Main Methods:
- Retrospective cohort study of 278 patients with nerve biopsies diagnostic or suggestive of vasculitis (2001-2020).
- Collected and analyzed clinical and histopathological data.
Main Results:
- Large-arteriole vasculitis showed more acute onset (50.4% vs 26.8%) and distal asymmetric polyneuropathy (48.0%).
- Microvasculitis had a chronic onset (57.5% vs 33.6%), longer diagnosis/plateau times, radiculoplexus neuropathy (43.8%), more autonomic involvement (24.2%), and association with non-systemic vasculitis (71%).
- Systemic autoimmune disease was more common in large-arteriole vasculitis (70.4% vs 22.9%).
Conclusions:
- Nerve large-arteriole vasculitis and microvasculitis exhibit distinct yet overlapping clinical presentations.
- Large-arteriole vasculitis typically presents acutely with distal polyneuropathy and systemic autoimmune associations.
- Microvasculitis often presents chronically with proximal nerve involvement, autonomic dysfunction, and non-systemic vasculitis.
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