Stroke Due to Vasculitis in Adults: An overview

1. A D E W I J A Y A , M D – M A Y 2 0 2 2
Stroke Due to Vasculitis in Adults
An Overview

2. Introduction
 Vasculitis: Inflammation of blood vessels and
inflammatory leukocytes in vessel walls.
 Increase both ischaemic and hemorrhagic stroke
 Ischaemic stroke: compromise of vessel lumina and
distal tissue ischemia
 Hemorrhagic stroke: loss of vessel integrity
Younger DS. Stroke due to Vasculitis in Children and Adults. Neurologic Clinics. 2019 May 1;37(2):279-302.

3. Classification
 Primary CNS vasculitis (PCNSV)
Granulomatous angiitis of the brain
Adult primary angiitis of the CNS (PACNS)
Childhood PACNS (cPACNS)
 Systemic vasculitis
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1–11.
Salvarani C, Brown RD Jr, Christianson TJH, et al. Adult primary central nervous system vasculitis: treatment and course. Arthritis Rheum 2015;67:1637–45.
Younger DS, Hays AP, Brust JCM, et al. Granulomatous angiitis of the brain. An inflammatory reaction of diverse etiology. Arch Neurol 1988;45:514–8.
Calabrese HL, Mallek JA. Primary angiitis of the central nervous system: report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 1988;67:20–39.

4. Primary Central Nervous System Vasculitis in Adults
 Persistent neurologic deficits, including stroke and
headache, were the commonest initial symptoms
affecting 68%
 Brain infarction typically bilateral and multiple,
cortical and subcortical involvement
 8 % intracranial hemorrhage
Calabrese HL, Mallek JA. Primary angiitis of the central nervous system: report of 8 new cases, review of the literature, and proposal for diagnostic criteria. Medicine 1988;67:20–39.
Salvarani C, Brown RD Jr, Calamia KT, et al. Primary central nervous system vasculitis of 101 patients. Ann Neurol 2007;62:442–51.
Birnbaum J, Hellmann DB. Primary angiitis of the central nervous system. Arch Neurol 2009;66:704–9

5. Primary Central Nervous System Vasculitis in Adults
 Angiography:
(64%) showed angiographic changes supporting the
diagnosis of PCNSV (manifesting areas of smooth-
wall segmental narrowing or dilatation, and
occlusions that affected multiple cerebral arteries
without the proximal vessel changes of
atherosclerosis or other causes)
(36%) showed CNS tissue changes of vasculitis
(demonstrating transmural vascular inflammation
involving leptomeningeal or parenchymal vessels)
Salvarani C, Brown RD Jr, Christianson TJH, et al. Adult primary central nervous system vasculitis: treatment and course. Arthritis Rheum 2015;67:1637–45

6. Systemic Vasculitis
Large vessel
• Giant cell arteritis
• Takayasu arteritis
Medium vessel
• Poliarteritis nodosa
Small vessel
• ANCA-associated vasculitis: GPA & PACNS
Younger DS. Stroke due to Vasculitis in Children and Adults. Neurologic Clinics. 2019 May 1;37(2):279-302.

7. Summary
 The vasculitides are diseases characterized by
inflammation of blood vessels and inflammatory
leukocytes in vessel walls
 There is an increased propensity for ischemic stroke
resulting from thrombosis and compromise of
vascular lumina
 Intracranial bleeding due to loss of vessel integrity

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