Vasculitic Peripheral Neuropathy, Differences Between Systemic and Non-Systemic Etiologies: A Case Series and Biopsy
J I Castiglione1, M Marrodan1, L Alessandro1
1Neurology department, FLENI, Buenos Aires, Argentina.
Systemic Vasculitic Neuropathy (SVN) and Non-Systemic Vasculitic Neuropathy (NSVN) share similar clinical signs, but erythrocyte sedimentation rate (ESR) can help differentiate them. Early nerve biopsy in untreated patients improves diagnostic accuracy for vasculitic peripheral neuropathy (VPN).
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Vasculitic peripheral neuropathy (VPN) results from inflammation of blood vessels affecting peripheral nerves.
- VPN is categorized as Systemic Vasculitic Neuropathy (SVN) when associated with systemic disease, or Non-Systemic Vasculitic Neuropathy (NSVN) when isolated to nerves.
Purpose of the Study:
- To compare clinical features, biopsy findings, and outcomes in patients diagnosed with VPN.
- To identify distinguishing factors between SVN and NSVN.
Main Methods:
- Retrospective review of adult VPN patient records from June 2002 to June 2019.
- Analysis of demographic data, clinical symptoms, nerve conduction studies, nerve biopsies, treatments, and clinical evolution.
- Inclusion criteria required at least 6 months of follow-up.
Main Results:
- No significant demographic or clinical differences were observed between SVN (n=10) and NSVN (n=15) groups.
- NSVN patients had a longer median delay from symptom onset to nerve biopsy (10 vs. 5.5 months).
- Elevated erythrocyte sedimentation rate (ESR > 20 mm/h) was more frequent in SVN (100% vs. 60%).
- Treatment-naive patients showed higher rates of active nerve biopsy lesions (92% vs. 38%) and definite VPN diagnoses (92% vs. 46%) compared to those pre-treated with corticosteroids.
Conclusions:
- While clinical presentations are similar, ESR is a valuable marker for differentiating SVN and NSVN.
- Prompt nerve biopsy in untreated patients enhances diagnostic accuracy for VPN, preventing misdiagnosis.
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