Vasculitic neuropathy (VN) involves the peripheral nervous system and can be isolated (NSVN) or part of a systemic disease (SVN). VN often presents as mononeuritis multiplex, characterized by asymmetric dysesthesias, paresthesias, and palsies. Nerve biopsy studies suggest NSVN accounts for about 28% of VN cases. VN can present in various forms, including distal sensorimotor polyneuropathy and plexopathy, and is often underdiagnosed due to its heterogeneous clinical phenotypes.

Diagnosis of VN involves a thorough medical history, physical and neurological examinations, and extensive testing to identify systemic involvement. Nerve conduction studies and EMG help reveal the neuropathy pattern and guide biopsy site selection, with nerve biopsy confirming the diagnosis. Treatment primarily involves immunosuppression, with glucocorticoid monotherapy for mild cases and combination therapy for aggressive or progressive NSVN. Early and aggressive treatment, along with physical rehabilitation, improves prognosis. Further research is needed to understand VN’s natural history, treatment, and pathogenic mechanisms.

Reference: Witt A, Kodal L, Dysgaard T. Diagnosis and Treatment of Vasculitic Neuropathy. Practical Neurology. Accessed May 21, 2024. https://practicalneurology.com/articles/2024-apr/diagnosis-and-treatment-of-vasculitic-neuropathy?c4src=home:feed