신경계를 침범하는 혈관염 증후군

Abstract

Background: The vasculitis are a group of diseases and disorders sharing the central feature of inflammation of the blood vessel wall with attendant tissue ischemia. The purpose of this study was to determine the types and frequency of neurological involvement in patients with vasculitis. Methods: We reviewed the medical records of 131 patients with vasculitis who were examined at Severance Hospital. Results: The nervous system was involved in 47 out of 131 cases. Patients with systemic necrotizing vasculitis showed the highest frequency of neurological involvement (78.3%) of which peripheral involvement was the most common. Patients with Wegener's granulomatosis showed 36.4% of neu-rological involvement of which all cases were cranial nerve palsy. Neurological involvement was found in 18.7% of cases with Takayasu's arteritis and was limited to the central nervous system. Neurological symptoms were initial mani-festations in 56.5% of cases with systemic necrotizing vasculitis, 9% with Wegener's granulomatosis, and 2.7% with Takayasu's arteritis. Among various laboratory values, positive rate of p-ANCA was significantly higher in Wegener's granulomatosis cases with neurological involvement than cases without involvement. Conclusions: We found that the frequency and distribution of neurological involvement vary with the underlying disorder. Neurological manifestations may provide an important clue for the diagnosis of systemic necrotizing vasculitis because neurological involvements frequently occur as an initial manifestation of it.