Nonsystemic Vasculitic Neuropathy-A Brazilian Case Series.
Victor Evangelista Rodrigues Pereira1, Roberto Pereira Santos1, Marcos Raimundo Gomes de Freitas1
1Hospital Universitário Clementino Fraga Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil.
Journal of the Peripheral Nervous System : JPNS
|December 9, 2025
Summary
Nonsystemic vasculitic neuropathy (NSVN) often presents as painful sensory or sensorimotor deficits, mimicking other neuropathies. Early diagnosis and nerve biopsy are crucial for managing this underrecognized condition to prevent significant morbidity.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Vasculitides are immune-mediated disorders affecting blood vessels.
- Nonsystemic vasculitic neuropathy (NSVN) affects peripheral nerves without systemic involvement.
- NSVN is underrecognized and challenging to diagnose due to varied symptoms.
Purpose of the Study:
- To characterize the clinical, neurophysiological, and histopathological features of NSVN.
- To describe NSVN in a Brazilian cohort.
- To improve understanding and diagnostic strategies for NSVN.
Main Methods:
- Cross-sectional, ambispective cohort study.
- Included patients with histopathologically confirmed isolated peripheral nerve vasculitis.
- Data collected via clinical evaluation, neurophysiology, and nerve biopsy.
Main Results:
- 14 patients (57% female, mean age 61) included.
- Most presented with subacute painful sensorimotor deficits (57%) and mononeuropathies (78%).
- Biopsies showed possible (43%) or probable (42%) vasculitis; definite vasculitis in 14%.
Conclusions:
- NSVN often presents with painful symptoms and can mimic progressive axonal polyneuropathies.
- Early recognition and nerve biopsy are critical due to potential morbidity.
- The variability and diagnostic complexity suggest NSVN is underdiagnosed.
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