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Padoan et al. Vessel Plus 2021;5:41 https://dx.doi.org/10.20517/2574-1209.2021.41 Page 13 of 18

in the outpatient clinic and probably the easiest way to histologically confirm diagnosis in GPA and EGPA.
Endoscopic sinus surgery should be indicated in GPA patients presenting with complications (e.g.,
mucoceles, fungal infections, and orbital/lacrimal pathways involvement). In cases unresponsive to medical
treatments, endoscopic sinus surgery should be cautiously considered in GPA patients, since recent
evidence suggests that sinus surgery is associated with osteitis progression and an increase in nasal space
[120]
and crust formation .
Reconstructive surgery in GPA (e.g., septal perforation or saddle nose repair) is controversial and needs
careful planning. Although no consensus exists on the best time to perform it, it should be indicated when
the disease is in complete remission, with Unadkat et al. suggesting waiting for further 6-12 months after
[121]
disease stabilization.

Surgical measures for symptoms relief are reserved for refractory otologic manifestations in GPA. Patients
with recurrent otitis media with persistent symptomatic middle ear effusions or eustachian tube dysfunction
[122]
may benefit from myringotomy tube placement . In the case of recurrent mastoiditis, mastoidectomy is
advisable. An external approach and/or endonasal procedures may be used to perform
dacryocystorhinostomy for epiphora and/or chronic infection in the lacrimal sac.

Although laryngo-tracheal manifestations of GPA are extremely rare (around 10%-15%), the subglottic
stenosis is the most frequently observed. Failure of glucocorticoids and immunosuppressive treatment in
symptoms relief is the main indication for surgical treatment of subglottic stenosis. Endoscopic intervention
or dilation are preferred. Treatment failures are relatively high, ranging from 49% after one year to 80% at
five years after the first procedure, according to a multicenter study including 47 patients .
[49]
Considering EGPA patients, the role of endoscopic sinus surgery is still a matter of debate, with a
[73]
controversial opinion reported in the literature . In the future, surgery in EGPA will probably collide with
the introduction of new monoclonal antibodies in the treatment regimens.

Surgical indications and proper timing of procedures is critical in AAV patients and should always be
planned in a multidisciplinary setting in conjunction with all the medical figures involved to avoid poor
outcomes and potential surgical complications.

CONCLUSIONS
ENT involvement in AAV, especially in GPA and EGPA, represents one of the most frequent symptoms.
Although patients with ENT symptoms have better survival and less renal involvement, they typically
experience persistent or relapsing disease together with long-term exposure to therapies, leading to
irreversible damage.

The burden of sinonasal morbidity on quality of life is significant and comparable to other common chronic
diseases, with an impairment especially of social functioning and well-being perception, perhaps as a result
of the stigma of constant purulent rhinorrhea, embarrassing epistaxis, or nasal deformity from cartilage
destruction [123,124] . The high impact of ENT symptoms on quality of life of AAV patients confirms the
importance of their early treatment through specific local and systemic approaches .
[124]

The otorhinolaryngologist is often one of the first physicians to see patients with GPA. The most frequent
clinical manifestation of GPA is related to ENT involvement, in all of its forms, which may be the first or
the only symptom. Thus, a close collaboration between the otorhinolaryngologist and rheumatologist is