Background/Purpose: Eosinophilic angiocentric fibrosis (EAF) is a rare localized fibro-inflammatory lesion involving usually upper respiratory tract and the orbit. It could mimic ENT manifestations of systemic vasculitis particularly GPA. The diagnosis is based on characteristic histologic findings with a mixed inflammatory infiltrate with eosinophils and fibrosis around small caliber arteries with « onion-skin » pattern. Some autors suggest that EAF is a part of the spectrum of IgG4-related systemic disease.

Methods: We report 3 cases of histology-proven EAF. Demographic, clinical, biological, radiologic and histological features are examined. The 3 patients had a 18-fluorodeoxyglucose positron emission tomography (FDG PET) and evaluation of circulating total plasmablast count (CD19low, CD38+, CD20-, CD27+).

Results: table Two females and one male patients were aged from 29 to 50 years. The disease involved the nasal cavity in two cases and the subglottic area in the third case. The demographic, biological and clinical features (table) were consistent with reports of EAF published previously. All cases demonstrated a dense fibrotic stroma with a perivascular onion skin type pattern and an inflammatory infiltrate with eosinophils, lymphocytes and plasma cells. No vasculitis, granuloma formation or necrosis were identified in any of these cases. All patients had a radiological evaluation with a computed tomography (CT) and a FDG-PET. CT evaluations showed expanding soft tissue masses into the maxillary sinus and anterior nasal cavity in 2 cases and soft tissue thickening in the sub-glotting region of the larynx in one case. There was bone destruction in one case. The FDG-PET showed an enhanced FDG uptake of the EAF lesion. No other abnormal hypermetabolic foci were detect on this exam. The serum IgG4 concentration was normal in the 3 cases (from 23 to 50 mg/dL). The circulating plasmablast count was increased in 2 cases (6728/mL et 2958/mL) and normal in the third patient (508/mL). Two patients received glucosteroids associated in one with colchicin without efficiency. Patients with sinonasal lesion underwent partial surgical excision with relapse in the 2 cases. Patient with the subglottic stenosis was improved by endoscopic dilatation.

Conclusion: EAF is an unrecognized entity mimicking a necrotizing vasculitis limited to upper respiratory tract. FDG PET confirms that EAF is a limited pathology without extra-ENT involvment. The anatomopathology examination by an experienced pathologist is the cornerstone of the diagnosis. Despite some differences, EAF share many characteritics with IgG4 related disease like clinical presentation, similar histologic pattern and increase of plasmablast count.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/eosinophilic-angiocentric-fibrosis-a-mimic-of-vasculitis-in-igg4-related-disease-spectrum/