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

Ammar Haikal; Tanawat Attachaipanich; Kyle Myers; Paul Schmidt; Arsany Anis

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

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

Authors

Ammar Haikal¹, Tanawat Attachaipanich², Kyle Myers¹, Paul Schmidt¹, Arsany Anis³

Affiliations

¹ Internal Medicine, University of Kansas Medical Center, Kansas City, USA.
² Internal Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, THA.
³ Internal Medicine, Saint Michael's Medical Center, Newark, USA.

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.

Publication types

Case Reports