Clinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China

Vasculitic neuropathy (VN) is a disease in which vessel inflammation happens and injures peripheral nerves. Despite increasing awareness, features of VN in mainland China are still understudied.

To characterize the clinical, pathological features and outcomes of VN in mainland China, and evaluate clinicopathological correlations: METHODS: We retrospectively reviewed records of VN patients diagnosed pathologically between June 1999 and December 2024, including demographic data, clinical manifestations, biopsy features, and outcomes.

112 patients were totally included. All presented with axonal sensorimotor neuropathy, most commonly involving the tibial (90.63%) and peroneal (87.50%) nerves. Systemic VN (SVN) showed more frequent transmural inflammatory cell infiltration (ICI) than non-systemic VN (NSVN) (p = 0.046). Eosinophilic granulomatosis with polyangiitis (EGPA) was more common in VN without ICI than VN with ICI (p = 0.008). In SVN, the ICI-positive exhibited more severe distal upper limb weakness (p = 0.042) and higher thrombosis rates (p = 0.001) than ICI-negative. Of the 63 patients followed, 62 received glucocorticoids with or without immunosuppressants. Thirteen died from multi-organ complications (12 SVN and 1 NSVN), while others achieved remission. The 5-year all-survival rate was 80.23% (95% CI 66.83%- 88.66%).

This first large cohort of VN in mainland China delineates its clinical-pathological features. EGPA showed a lower diagnostic yield on biopsy, suggesting diverse mechanisms of vascular injury. Nerve biopsy remains the diagnostic gold standard. The overall prognosis of VN is relatively favorable, emphasizing the need for early recognition and treatment.