Eosinophilic Angiocentric Fibrosis of the Subglottis

Objectives:Eosinophlic angiocentric fibrosis (EAF) is a rare disorder that belongs to the spectrum of IgG4-related diseases (IgG4-RD). We review a case of EAF presenting as a subglottic stenosis.Methods:We discuss the pertinent clinical, radiographic, and histological findings, demonstrating the diagnostic challenges associated with EAF. We review the literature on respiratory tract EAF and discuss the associated management dilemmas when this otherwise benign disease affects critical structures. A 44 year-old female presented a twelve-month history of progressive dyspnea and stridor. A pulmonary function test (PFT) showed flattened inspiratory and expiratory flow loops suggestive of subglottic stenosis. Pre-operative computed tomography (CT) revealed a subglottic mass with cricoid erosion. During endoscopy, a submucosal lesion was noted to involve the conus elasticus and cricoid. The airway was temporized with CO2 laser resection of the mass and dilation.Results:Histology showed an inflammatory infiltrate...