Primary central nervous system vasculitis with intracranial aneurysm.

Autor: Salvarani C; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA; Rheumatology Division, Azienda USL-IRCCS di Reggio Emilia e Università di Modena e Reggio Emilia, Reggio Emilia, Italy. Electronic address: carlo.salvarani@ausl.re.it., Brown RD Jr; Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA., Christianson TJH; Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, Minnesota, USA., Huston J 3rd; Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA., Giannini C; Department of Anatomic Pathology, Mayo Clinic, Rochester, Minnesota, USA., Hunder GG; Division of Rheumatology, Mayo Clinic, Rochester, Minnesota, USA.
Jazyk: angličtina
Zdroj: Seminars in arthritis and rheumatism [Semin Arthritis Rheum] 2024 Oct; Vol. 68, pp. 152506. Date of Electronic Publication: 2024 Jun 29.
DOI: 10.1016/j.semarthrit.2024.152506
Abstrakt: Background: Unruptured intracranial aneurysms (UIAs) are rarely reported in primary central nervous system vasculitis (PCNSV). In this study we described the clinical findings, response to therapy, and outcomes of UIA in a large cohort of PCNSV patients.
Methods: We retrospectively studied 216 consecutive patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 40-year period. 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.
Results: 12/216 (5.5 %) PCNSV patients had at least one UIA. Two patients underwent biopsies; one yielded negative results, while the other showed necrotizing vasculitis. Eleven patients had evidence of UIA on angiogram at diagnosis. One patient developed an aneurysm during the follow-up associated 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 < 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 (median: 18.5 months; range 1-151 months) 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).
Conclusions: UIAs uncommonly occur in PCNSV.
Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
(Copyright © 2024 Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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