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Page 2 of 8 Hegazy et al. Vessel Plus 2021;5:46 https://dx.doi.org/10.20517/2574-1209.2021.52

INTRODUCTION
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous inflammation with necrotizing
vasculitis that involves small- and medium-sized blood vessels. It is one of the anti-neutrophil cytoplasmic
antibody (ANCA)-associated vasculitides that commonly involves the upper and lower respiratory tract and
the kidneys. Ear, nose, and throat (ENT) symptoms represent the most frequent manifestations at the onset
of GPA. Their presence can also occur in a milder GPA subset with a better outcome regarding lower renal
involvement and lower mortality rate, irrespective of the ANCA status. They can also, not infrequently,
[1]
coexist with lung nodules .

The burden of diagnosing a limited disease with localized manifestations in the upper aerodigestive tract
rests mainly with the otolaryngologist. The persistence of sino-nasal symptoms and inadequate response to
the standard of care should prompt surgeons to consider a spectrum of diseases including GPA. Thus, the
diagnosis of the limited form of GPA is often challenging leading to a delay in the start of treatment. This
could lead to major sequelae due to tissue destruction .
[2]

ENT surgeons play a key role in early GPA diagnosis and treatment in collaboration with rheumatologists,
pulmonologists, and nephrologists if needed, especially in GPA cases with negative ANCA antibodies, the
diagnosis of which is often more challenging and delayed . Limited forms of GPA predominantly affect the
[3]
upper respiratory tract, whereas generalized forms mostly include lower respiratory tract, kidney, and other
systems .
[4]
Clinical picture
Approximately 63% of GPA patients present with rhinogenic, otologic, or laryngopharyngeal
manifestations. Individually, rhinogenic, otologic, and laryngopharyngeal manifestations represent 41%,
16%, and 6% of GPA manifestations, respectively . Of GPA patients undergoing ENT evaluation, 89% have
[5]
[6]
sino-nasal involvement . Clinical presentation is broad and includes an array of non-specific symptoms
that embrace, though are not limited to, the following [1,7-15] :

- Symptoms of chronic sinusitis are the most common initial complaint in GPA. Sino-nasal involvement in
GPA is often misdiagnosed as chronic sinusitis or rhinitis that fails to respond to the initial conventional
therapy.

- Bloody nasal discharge with crusting.

- Sino-nasal masses.

- Oral cavity: purplish hyperplastic gingival lesions, teeth loosening, and failure of oral wounds to heal.

- Strawberry gingival hyperplasia.

- Deformities as a result of bony destruction such as septal perforation (mainly posterior) and saddle nose
deformity, which may develop from the involvement of Haversian canals.

- Stridor, possibly leading to respiratory compromise, from tracheal or subglottic granulomatous masses.
The sub-glottis is the most affected laryngeal subunit. Between 10% to 20% of GPA patients acquire
subglottic stenosis . Extension of inflammation to the glottis leads to the development of dysphonia. Also,
[16]
[17]
hemoptysis, dyspnea, stridor, and wheezing are possible presentations .