Early ENT Manifestations in Eosinophilic Granulomatosis with Poliangioitis: Results from Our Cohort Group and Literature Review

Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic necrotizing vasculitis, affecting small-to-medium sized vessels. EGPA’s clinical manifestations are heterogeneous affecting different organs and systems and the upper respiratory tract can be affected with Ear, Nose and Throat (ENT) involvement. The aim of our study was to assess type manifestations at time of diagnosis in a cohort of EGPA patients and correlate findings with baseline variables (sex, age, antineutrophil cytoplasmic antibodies –ANCA- status) and literature reports. Main ENT manifestations in our patients at time of diagnosis were: nasal polyposis (CRSwNP) (52%), turbinate hypertrophy (48%), nasal swelling (40%), rhinorrhoea (40%), chronic rhinosinusitis without nasal polyposis (CRSsNP) (32%), nasal bone deformities (32%), nasal crusts (20%), nasal mucosal ulcers (12%), corditis (12%), hoarseness/dysphonia (12%), hearing loss (12%), mucoceles(4%) eosinophilic rhinitis (4%). No correlations were found between sex, age, ANCA status and ENT clinical manifestations. A polymorphic ENT involvement is often observed at early stages of EGPA. The presence of nasal, sinus, ear and/or laryngeal manifestations in patients with asthma and hypereosinophilia, independently of sex, age or ANCA status, should raise allert for further investigation and differential diagnosis for EGPA. ENT specialists should be aware of their leading position in this diagnostic race.