Page 27 - Read Online

Padoan et al. Vessel Plus 2021;5:41 https://dx.doi.org/10.20517/2574-1209.2021.41 Page 9 of 18

[76]
obtained under general anesthesia . Recent data demonstrate that, alongside the well-known eosinophil-
rich inflammation, there may be other cells contributing to the inflammatory process, such as
[28]
neutrophils , but specific markers are still lacking.

RADIOLOGICAL FINDINGS
Performing CT or MRI scans may be used and recommended on an individual basis, according to the
location of the involvement and the clinical manifestations.

[77]
In a systematic review carried out on sinus imaging findings in GPA, 92.6% of the patients had
abnormalities on sinus CT: mucosal thickening of the paranasal sinuses and nasal fossae (87.7%), bony
destruction (59.9%), and osteoneogenesis with foci of sclerosing osteitis and bone thickening (46%-59%)
[Figure 3]. Bony obliteration of sinuses is relatively rare. Septal erosion was observed in 59.4% of the
patients and 27.1% had orbital involvement. MRI imaging showed similar rates of mucosal thickening
(89.9%) and granulomas in 14.5% of the patients, while conversely bony erosion was reported in only 10.1%
of the cases. In EGPA patients, mucosal thickening, nasal polyps, and pansinusitis are commonly reported,
while bony destruction is absent . An alternative diagnosis, rather than AAV, should be suspected in the
[78]
presence of bone erosion of the hard palate or the maxillary wall and alveolus. However, even though CT or
MRI features are often non-specific, imaging might improve management of AAV patients, for example by
quantifying the extent of sinus involvement.

Finally, CT and MRI scans can also be used to better define lesions in subglottic/tracheal stenosis. These
tests are also suggested for patients with ear involvement who are refractory to treatment or in cases of
cranial nerve palsy .
[79]

DIFFERENTIAL DIAGNOSIS
Many of the clinical features of AAV are non-specific, and, thus, the potential differential diagnoses are
several. In the presence of ulcerative lesions of the ENT region, AAV should be included in the differential
diagnosis; in addition, infections, inflammatory autoimmune diseases, malignancies, and substance abuse
conditions can also cause granulomatous inflammation, which may lead to extensive damage [Table 2].
Complete and careful examination is warranted, as well as looking for evidence of different organ
involvement other than the head and neck region. Moreover, all patients presenting with ENT symptoms
resembling GPA should be evaluated with flexible endoscopy and imaging (CT and/or MRI) in order to
quantify disease extent and identify the best area to biopsy. Additionally, serologic assessment should be
performed; firstly, ANCA testing can lead to the proper diagnosis. An increasing presence of clinical,
serological, and histological factors should enforce clinical suspicion of AAV, if there are no signs or
symptoms that lead to a different diagnosis.

TREATMENT OPTIONS
Local treatment
Tissue damage caused by inflammation represents one of the major sources of morbidity for patients with
AAV and ENT involvement. Some of these symptoms need adequate treatments, administered alone or in
combination with systemic medical treatment, such as surgical or endoscopic repair or the delivery of
topical or injectable medications directly to the site of disease .
[80]

Sinonasal symptoms can be relieved with vigorous nasal irrigation and topical medications applied directly
to the nasal mucosa. This can be achieved in combination with glucocorticoids. Nasal irrigation with saline
on a regular basis can help to dissolve crusts that can become a pablum for bacterial proliferation and block