This first large cohort of vasculitic neuropathy in mainland China delineates its clinical-pathological features, finding that nerve biopsy remains the diagnostic gold standard and that overall prognosis is relatively favorable with a 5-year all-survival rate of 80.23%.
Key Findings
Results
All 112 VN patients presented with axonal sensorimotor neuropathy, most commonly involving the tibial and peroneal nerves.
Total cohort included 112 patients diagnosed pathologically between June 1999 and December 2024 at a single center in mainland China.
Tibial nerve involvement was present in 90.63% of patients.
Peroneal nerve involvement was present in 87.50% of patients.
The study was retrospective in design, reviewing demographic data, clinical manifestations, biopsy features, and outcomes.
Results
Systemic VN (SVN) showed more frequent transmural inflammatory cell infiltration (ICI) than non-systemic VN (NSVN).
The difference in transmural ICI frequency between SVN and NSVN was statistically significant (p = 0.046).
ICI refers to inflammatory cell infiltration identified on nerve biopsy specimens.
This finding highlights a pathological distinction between systemic and non-systemic forms of vasculitic neuropathy.
Results
Eosinophilic granulomatosis with polyangiitis (EGPA) was more common in VN without ICI than VN with ICI.
The association between EGPA and absence of ICI on biopsy was statistically significant (p = 0.008).
The authors interpreted this as suggesting EGPA has a lower diagnostic yield on biopsy.
This finding suggests diverse mechanisms of vascular injury in EGPA compared to other forms of VN.
Results
In SVN, ICI-positive patients exhibited more severe distal upper limb weakness and higher thrombosis rates than ICI-negative patients.
More severe distal upper limb weakness in ICI-positive SVN compared to ICI-negative SVN was significant (p = 0.042).
Higher thrombosis rates in ICI-positive SVN compared to ICI-negative SVN was significant (p = 0.001).
These clinicopathological correlations were identified within the SVN subgroup specifically.
Results
The 5-year all-survival rate for VN patients was 80.23%.
The 5-year all-survival rate was 80.23% (95% CI 66.83%–88.66%).
Of 63 patients followed, 62 received glucocorticoids with or without immunosuppressants.
Thirteen patients died from multi-organ complications, comprising 12 SVN and 1 NSVN patient.
Patients who did not die achieved remission.
The authors characterized the overall prognosis of VN as 'relatively favorable.'
Results
Nerve biopsy was identified as the diagnostic gold standard for vasculitic neuropathy.
All patients were diagnosed pathologically via nerve biopsy.
The diagnostic period spanned June 1999 to December 2024.
EGPA's lower diagnostic yield on biopsy (due to absent ICI) was noted as a caveat to biopsy interpretation.
The authors emphasized that nerve biopsy remains the gold standard despite variable pathological findings across VN subtypes.
Zhou X, Shan D, Wu B, Liu F, Zhang D, Lin P, et al.. (2026). Clinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China.. European journal of neurology. https://doi.org/10.1111/ene.70540