Distinctive clinical features in biopsy-proven nerve large-arteriole vasculitis and microvasculitis
Issued Date
2025-03-06
Resource Type
eISSN
14602156
Scopus ID
2-s2.0-86000722454
Pubmed ID
39715342
Journal Title
Brain : a journal of neurology
Volume
148
Issue
3
Start Page
1031
End Page
1042
Rights Holder(s)
SCOPUS
Bibliographic Citation
Brain : a journal of neurology Vol.148 No.3 (2025) , 1031-1042
Suggested Citation
Soontrapa P., Pinto M.V., Shouman K., Mandrekar J., Engelstad J.N.K., Aragon Pinto C., Taylor S., Mauermann M.L., Berini S.E., Bosch E.P., Rubin D.I., Koster M.J., Weyand C.M., Warrington K.J., Klein C.J., Dyck P.J., Dyck P.J.B. Distinctive clinical features in biopsy-proven nerve large-arteriole vasculitis and microvasculitis. Brain : a journal of neurology Vol.148 No.3 (2025) , 1031-1042. 1042. doi:10.1093/brain/awae406 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/106786
Title
Distinctive clinical features in biopsy-proven nerve large-arteriole vasculitis and microvasculitis
Corresponding Author(s)
Other Contributor(s)
Abstract
Vasculitic neuropathy is caused by inflammatory destruction of nerve blood vessels resulting in nerve ischaemia. Nerve vasculitis can be divided into two categories based on vessel size: large-arteriole vasculitis (≥75 µm) and microvasculitis (<75 µm). Herein, we characterize the clinical features of nerve large-arteriole vasculitis in comparison to nerve microvasculitis. This is a retrospective cohort study of patients evaluated and biopsied at Mayo sites between 2001 and 2020. We collected clinical and histopathological data from patients whose nerve biopsies were either diagnostic or highly suggestive of nerve vasculitis. Two hundred and seventy-eight cases were identified: 125 cases of large-arteriole vasculitis and 153 cases of microvasculitis. Nerve large-arteriole vasculitis presented with a more acute (50.4% versus 26.8%) versus chronic onset (33.6% versus 57.5%) than nerve microvasculitis (P = 0.0001). Nerve microvasculitis had longer mean time to diagnosis (10.5 versus 4.3 months; P < 0.0001) and longer time to plateau (8.9 versus 3.5 months; P < 0.0001). Nerve large-arteriole vasculitis typically presented as distal asymmetric polyneuropathy (48.0%), whereas nerve microvasculitis typically presented as radiculoplexus neuropathy/polyradiculoneuropathy (more proximal involvement of shoulder and thigh) (43.8%) (P < 0.0001). Systemic autoimmune disease was more common in nerve large-arteriole vasculitis (70.4% versus 22.9%, odds ratio, 8.0; 95% confidence interval, 4.7-13.7; P < 0.0001). Nerve microvasculitis was significantly related to non-systemic vasculitis (71% versus 23%, odds ratio, 7.9; 95% confidence interval, 4.6-13.6; P < 0.0001). Nerve microvasculitis had more autonomic involvement (24.2% versus 7.2%, odds ratio, 4.1; 95% confidence interval, 1.9-8.9; P = 0.0002). Nerve large-arteriole vasculitis and nerve microvasculitis have different but overlapping clinical features. Nerve large-arteriole vasculitis usually presents with acute onset, distal asymmetric polyneuropathy, associated with other autoimmune diseases and systemic involvement. In contrast, nerve microvasculitis usually presents with a subacute/chronic onset, as radiculoplexus neuropathy/polyradiculopathy (distal and proximal pattern) with autonomic involvement, and is more often a form of non-systemic vasculitis.