Pathology of Primary Angiitis of the Central Nervous System

Selima Siala; Nabil Rahoui; Benjamin Cho; Carlos A Zamora

doi:10.1016/j.nic.2023.06.002

Pathology of Primary Angiitis of the Central Nervous System

Neuroimaging Clin N Am. 2024 Feb;34(1):31-37. doi: 10.1016/j.nic.2023.06.002. Epub 2023 Jul 30.

Authors

Selima Siala¹, Nabil Rahoui², Benjamin Cho², Carlos A Zamora³

Affiliations

¹ Department of Radiology, University of North Carolina School of Medicine, 101 Manning Drive, Chapel Hill, NC 27514, USA. Electronic address: selima_siala@med.unc.edu.
² Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, 101 Manning Drive, Chapel Hill, NC 27514, USA.
³ Department of Radiology, University of North Carolina School of Medicine, 101 Manning Drive, Chapel Hill, NC 27514, USA.

PMID: 37951703
DOI: 10.1016/j.nic.2023.06.002

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare and potentially severe form of vasculitis that is limited to the brain, spinal cord, and meninges. Despite extensive research, the etiology and underlying immunologic mechanisms of PACNS remain largely unknown. PACNS presents with a variety of clinical, radiological, and pathologic features, but it is generally characterized by inflammation and destruction of the walls of blood vessels in the CNS, which can lead to tissue ischemia and/or hemorrhage. Three main histopathologic patterns have been identified, namely granulomatous, lymphocytic, and necrotizing vasculitis.

Keywords: Biopsy; Diagnostic criteria; Primary angiitis of the central nervous system (PACNS); Vasculitis.

Publication types

Review

MeSH terms

Brain / pathology
Humans
Spinal Cord / diagnostic imaging
Spinal Cord / pathology
Vasculitis, Central Nervous System* / diagnostic imaging
Vasculitis, Central Nervous System* / pathology

Supplementary concepts

Primary angiitis of the central nervous system