OP0196 PRIMARY CENTRAL NERVOUS SYSTEM VASCULITIS WITH INTRACRANIAL ANEURYSM
Authors: C. Salvarani, R. Brown, T. Christianson, J. Huston, C. Giannini, G. Hunder
Journal: Annals of the Rheumatic Diseases (2024)

<h3>Background:</h3> Unruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). <h3>Objectives:</h3> In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients. We also compared PCNSV patients with and without a co-occurring UIA. <h3>Methods:</h3> Retrospective single-center study utilizing a cohort of 216 consecutive patients with PCNSV who were evaluated at the Mayo Clinic over a 40-year period. The diagnosis of PCNSV was based on brain or spinal cord biopsy, cerebral angiography or both. UIAs were identified on cerebral angiography. The clinical, laboratory, radiologic and pathologic findings, management, and outcomes of patients with UIA were described and compared with those without UIA. <h3>Results:</h3> 12/216 (5.5%) patients with PCNSV had at least one UIA. Biopsy was positive in one patient and showed necrotizing vasculitis. Eleven patients had evidence of UIA on angiogram at diagnosis. One patient developed an aneurysm during the follow-up in association with a worsening of vasculitic radiological findings. The most common presenting symptom for PCNSV in the setting of UIA was headache (67%), followed by persistent neurologic deficit or stroke (50%). Most patients with UIA presented with multiple cerebral infarcts on MRI (67%), one patient had subarachnoid hemorrhage, and one left parieto-occipital intracerebral hematoma, both unrelated to the aneurysm. Black blood imaging was performed in 4 patients and 2 showed segmental circumferential mural enhancement involving multiple vessels. Two patients had 2 UIAs, while the other 10 had 1. The most frequent UIA location was internal carotid artery (50%), followed by anterior cerebral artery (21%). Ten of the UIAs were &lt; 5 mm in diameter, and 3 were 5-7 mm in diameter; the size was not available for one. All UIAs were unchanged in size and configuration during follow-up and no new aneurysms were detected. Compared to the 204 patients with PCNSV without a UIA, no significant clinical differences were observed, except for a reduced disability at last follow-up (p = 0.038). An increased survival trend for UIA patients was also observed: at the end of follow-up no patients with aneurysms died, compared to 46 (22.5%) of the 204 patients without UIA (p = 0.09). <h3>Conclusion:</h3> Intracranial aneurysms uncommonly occur in PCNSV. Patients with UIA in the setting of PCNSV had a good prognosis in this small series of patients with co-occurring UIA and PCNSV. <h3>REFERENCES:</h3> [1] Salvarani C, Brown RD Jr, Hunder GG. Adult primary central nervous system vasculitis. Lancet 2012; 380:767–777. [2] Brown RD Jr, Broderick JP. Unruptured intracranial aneurysms: epidemiology, natural history, management options, and familial screening. Lancet Neurol 2014; 13:393-404. <b>Table 1.</b> <h3>Acknowledgements:</h3> <b>NIL.</b> <h3>Disclosure of Interests:</h3> <b>None declared.</b>