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Page 4 of 18 Padoan et al. Vessel Plus 2021;5:41 https://dx.doi.org/10.20517/2574-1209.2021.41

Figure 2. Clinical features of granulomatosis with polyangiitis manifestations in the ENT region. Frontal and sagittal view of saddle-nose
deformity (A, B). Nasal endoscopy (C) showing subtotal septal perforation, bone erosion of the right middle and inferior turbinates, and
diffuse crusting covering the nasal mucosa. Endoscopic view of a concentric subglottic stenosis (D).

epistaxis and purulent nasal discharge.

A significant proportion of EGPA patients suffer from ENT symptoms, usually manifesting as allergic
rhinitis and chronic rhinosinusitis with or without polyps . Olsen et al. , in a series of 32 patients with
[25]
[26]
EGPA, reported nasal disease in 69% of the cases, nasal polyps in 50%, and nasal crusting in 36.3%. They
also found pansinusitis in 80% of patients. Another study on 28 EGPA patients demonstrated that ENT
[25]
involvement was present in 75% of the cases, with allergic rhinitis and nasal polyposis as the most frequently
observed manifestations at disease diagnosis, being observed in 42.8% and 76.1% of the patients,
respectively. A history of chronic rhinosinusitis was present in 14.2% of the subjects. Chronic rhinosinusitis
with diffuse and bilateral nasal polyps in EGPA is characterized by intense eosinophil tissue infiltration and
[27]
a chronic-relapsing course in almost one-third of cases, despite surgery and medical treatment . However,
in EGPA nasal polyps, in addition to tissue eosinophil aggregates, a diffuse neutrophilic infiltration can be
observed, supporting the hypothesis of neutrophils’ entanglement in the inflammatory process, even in the
absence of histological signs of vasculitis [28-30] . The role of neutrophil infiltrate in the nasal mucosa might be
to amplify eosinophil tissue recruitment, in addition to contributing to inflammation , and thus leading to
[28]
a more refractory manifestation.