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Rituximab for primary angiitis of the central nervous system: report of 2 patients from the French COVAC cohort and review of the literature. J Rheumatol 2013; 40:2102. 62. Salvarani C, Brown RD Jr, Huston J 3rd, et al. Treatment of primary CNS vasculitis with rituximab: case report. Neurology 2014; 82:1287. 63. Schuster S, Ozga AK, Stellmann JP, et al. Relapse rates and long-term outcome in primary angiitis of the central nervous system. J Neurol 2019; 266:1481. Topic 8230 Version 31.0 https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 26/31 7/7/23, 11:18 AM Primary angiitis of the central nervous system in adults - UpToDate GRAPHICS Carotid angiograms in primary angiitis of the central nervous system Left panel: An anteroposterior view of a right carotid angiogram in a patient with primary angiitis of the central nervous system (PACNS) reveals focal narrowing of multiple vessels in both the anterior and posterior systems. Right panel: A lateral view of a right carotid angiogram in PACNS demonstrates beading and multiple irregularities both proximally and distally. Courtesy of Leonard H Sigal, MD. Graphic 52006 Version 2.0 https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 27/31 7/7/23, 11:18 AM Primary angiitis of the central nervous system in adults - UpToDate Normal internal carotid artery angiogram An angiogram showing the normal radiographic appearance of the internal carotid artery and its branches. The internal carotid enters the skull at the carotid foramen, passes anteromedially, and then rises vertically (C6) to pass forward opposite the sella turcica (C4). The artery then passes upward within the dura (C3) and emerges below and medial to the anterior clinoid (C2), giving rise to the ophthalmic artery. The carotid then bifurcates (C1) to form the anterior and middle cerebral arteries. The anterior cerebral artery is directed anteromedially (A1) until it reaches the midline, where it turns upward (A2) to be joined by the anterior communicating artery. The middle cerebral artery extends laterally (M1). Courtesy of Leonard H Sigal, MD. Graphic 63788 Version 3.0 https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 28/31 7/7/23, 11:18 AM Primary angiitis of the central nervous system in adults - UpToDate Cerebral angiography of a patient with reversible cerebral vasoconstriction syndrome Note the multiple areas of stenosis (arrows) and dilatation in multiple vessels (arrowheads) of the M2 branch of the middle cerebral artery (A) and their resolution after one month (B). Reproduced with permission from: Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum 2002; 47:662. Copyright 2002 John Wiley & Sons, Inc. Graphic 66139 Version 4.0 https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 29/31 7/7/23, 11:18 AM Primary angiitis of the central nervous system in adults - UpToDate Histopathologic findings of primary angiitis of the CNS Histopathologic findings of a patient with primary angiitis of the central nervous system (PACNS). Granuloma changes are present in the vessel wall. Reproduced from: Hajj-Ali RA, Langford CA. Primary angiitis of the central nervous system. In: Kelley's Textbook of Rheumatology, 9th e GS, Budd RC, Gabriel SE, et al (Eds), Saunders, Philadelphia 2013. Vol 2, p.1519. Illustration used with the permission of Elsevier Inc. All reserved. Graphic 101671 Version 2.0 https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 30/31 7/7/23, 11:18 AM Primary angiitis of the central nervous system in adults - UpToDate Contributor Disclosures Rula A Hajj-Ali, MD Consultant/Advisory Boards: GlaxoSmithKline [EGPA]. All of the relevant financial relationships listed have been mitigated. Leonard H Calabrese, DO No relevant financial relationship(s) with ineligible companies to disclose. Gene G Hunder, MD No relevant financial relationship(s) with ineligible companies to disclose. Scott E Kasner, MD Grant/Research/Clinical Trial Support: Bayer [Stroke]; Bristol Meyers Squibb [Stroke]; Medtronic [Stroke]; WL Gore and Associates [Stroke]. Consultant/Advisory Boards: Abbvie [Stroke]; AstraZeneca [Stroke]; BMS [Stroke]; Diamedica [Stroke]; Medtronic [Stroke]. All of the relevant financial relationships listed have been mitigated. Philip Seo, MD, MHS No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system-in-adults/print 31/31