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 by ear, nose and throat (ENT) involvement. The aim of our study was to assess type manifestations at the 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. The main ENT manifestations in our patients at the time of diagnosis were: chronic rhinosinusitis with nasal polyposis (CRSwNP) (52%), turbinate hypertrophy (48%), nasal swelling (40%), rhinorrhea (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%) and eosinophilic rhinitis (4%). No correlations were found between sex, age, ANCA status and ENT clinical manifestations. A polymorphic ENT involvement is often observed in the 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 an alert for further investigation and differential diagnosis for EGPA. ENT specialists should be aware of their leading position in this diagnostic race.

Background/Objectives: Dupilumab is a human monoclonal antibody that targets both IL-4 and IL-13 signaling. Eosinophilia has been reported as a potential adverse event in treated patients in randomized controlled trials and 12-month real-life studies. This real-life, 24-month prospective study investigated the prevalence of eosinophilia, its consequences, and the effectiveness of dupilumab in a cohort of patients with severe asthma, chronic rhinosinusitis with nasal polyps, and atopic dermatitis. Methods: A total of 66 adult patients treated with dupilumab were included in this study. ACT, SNOT-22, and Smell-VAS, EASI, and absolute blood eosinophil count (AEC) were assessed according to the type of diagnosis at baseline (T0), after 6 (T6), 12 (T12), 18 (T18), and 24 (T24) months post-dupilumab initiation. Results: All patients experienced significant improvement in both symptoms and disease control following dupilumab treatment. A total of 27 out of 66 (40.9%) patients developed eosinophilia within six months of treatment (AEC T6 mean ± SD 0.67 ± 0.68 10⁹/L). Eosinophilia was generally mild and lasted on average for six months (AEC T12 mean ± SD 0.66 ± 0.65 10⁹/L) with AEC normalization after 18 months of treatment (AEC T18 mean ± SD 0.58 ± 0.48 10⁹/L). Ten patients (15.15%) developed hypereosinophilia, but no symptoms or signs of eosinophilic-related organ damage have been observed, with no need for dupilumab discontinuation. Conclusions: Dupilumab-related eosinophilia was common, generally mild, and transient, whereas persistent hypereosinophilia occurred in a small group of patients in the absence of symptoms or signs of eosinophilic damage.

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.