Central nervous system involvement in patients with antineutrophil cytoplasmic antibody–associated vasculitis: a study of 29 cases in a single Chinese center

Abstract

Background In antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV), central nervous system (CNS) involvement is relatively uncommon. The current study retrospectively investigated the clinical features and outcomes of AAV patients with CNS involvement. Methods A total of 497 AAV patients were retrospectively recruited in our center, twenty-nine of which had CNS involvement. Clinical and radiological manifestations and the outcomes of these patients were analyzed. Results The predominant symptom was sensorimotor impairment. According to the MRI findings, twenty-four patients had cerebral ischemic lesions, four patients had hemorrhagic lesions, and one patient had pituitary mass. With a median follow-up of 25 (range 9–45) months, 23 of 24 patients with cerebral ischemic lesions responded to induction therapy, and symptoms were ameliorated. The remaining one died from acute myocardial infarction 2 months after the diagnosis of cerebral ischemic lesions. Compared with patients without CNS involvement, patients with CNS involvement had significantly higher level of Birmingham Vasculitis Activity Score (23.5 ± 5.3 versus 18.8 ± 6.5, P < 0.01) and significantly higher proportion of peripheral nervous system involvement (58.6% versus 14.6%, P < 0.01). However, we did not found significant difference of patients’ survival between those with and without CNS involvement. Conclusion CNS involvement in Chinese patients with AAV was mainly manifested as cerebral ischemic lesions. Compared with patients without CNS involvement, patients with CNS involvement had a significantly more active disease of AAV, and significantly higher proportion of peripheral nervous system involvement. Key Points • CNS involvement in Chinese patients with AAV was mainly manifested as cerebral ischemic lesions. • Patients with CNS involvement had a significantly more active disease of AAV.