A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease.
anca-associated cns vasculitis
chronic hypertrophic pachymeningitis
interstitial lung disease
leptomeningeal enhancement
rituximab
Journal
Cureus
ISSN: 2168-8184
Titre abrégé: Cureus
Pays: United States
ID NLM: 101596737
Informations de publication
Date de publication:
29 Feb 2020
29 Feb 2020
Historique:
entrez:
8
4
2020
pubmed:
8
4
2020
medline:
8
4
2020
Statut:
epublish
Résumé
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).
Identifiants
pubmed: 32257689
doi: 10.7759/cureus.7144
pmc: PMC7105267
doi:
Types de publication
Case Reports
Langues
eng
Pagination
e7144Informations de copyright
Copyright © 2020, Haikal et al.
Déclaration de conflit d'intérêts
The authors have declared that no competing interests exist.
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