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Case Reports
. 2020 Feb 29;12(2):e7144.
doi: 10.7759/cureus.7144.

A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease

Ammar Haikal  1 , Tanawat Attachaipanich  2 , Kyle Myers  1 , Paul Schmidt  1 , Arsany Anis  3
Affiliations
Case Reports

A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease

Ammar Haikal et al. Cureus. .

Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing inflammation of the small vessels. Central nervous system (CNS) ANCA-associated vasculitis is a rare manifestation of AAV. Three mechanisms of AAV affecting the CNS have been reported which include contiguous granulomatous invasion from nasal and paranasal sinuses, remote granulomatous lesions, and vasculitis of small vessels. Chronic hypertrophic pachymeningitis (CHP) is the meningeal-site involvement in AAV caused by granulomatous inflammation in the dura mater. We present a case of pachymeningitis manifested with slowly progressive cognitive dysfunction, leptomeningeal enhancement on MRI, and necrotic vessels with surrounding inflammation on biopsy. This case represents a rare development of subsequent CNS AAV in a patient with ANCA-associated interstitial lung disease treated with rituximab with a resolution of leptomeningeal enhancement on a follow-up magnetic resonance imaging (MRI).

Keywords: anca-associated cns vasculitis; chronic hypertrophic pachymeningitis; interstitial lung disease; leptomeningeal enhancement; rituximab.

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Conflict of interest statement

The authors have declared that no competing interests exist.

Figures

Figure 1
Figure 1. Theleptomeninges showed some white-yellow thickening grossly
Figure 2
Figure 2. High power with a vessel and adjacent inflammatory cells
Figure 3
Figure 3. +Cluster of differentiation (CD) 68 macrophages
Figure 4
Figure 4. +Cluster of differentiation (CD) 3 positive T-cells
See this image and copyright information in PMC

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