Early Ear, Nose and Throat 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 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.

Keywords: ANCA; CRSwNP; EGPA; ENT; eosinophilic granulomatosis with polyangiitis; hearing loss; otitis; rhinosinusitis; vasculitis.

Figure 1

ENT manifestations at the time of diagnosis in our EGPA patients, divided by BVAS area.

Figure 2

(a) Nasal polyposis in a 35-year-old male EGPA patient. (b,c) Endoscopic image of nasal polyposis in the left and right nostrils.

Figure 3

Head CT scan. (a) Total obliteration of the mastoid cells (red arrows) by a hyperdense phlogistic tissue. Total obstruction of sphenoidal and subtotal obstruction of etmoid sinuses; (b) hypertrophy of the maxillary sinus mucosa. Absence of the right inferior turbinate (asportation by previous surgery).

Figure 4

Hearing loss and sex correlation.

Figure 5

(a) Eosinophils with abundant degranulation are observed in the nasal citology findings in a patient with eosinophilic rhinitis (May Grunwald-Giemsa (MGG), 1000×); (b,c) histological image of nasal polyp with eosinophilic infiltration at various magnifications (20× and 40×).