Page 54 - Read Online
P. 54
Page 4 of 8 Hegazy et al. Vessel Plus 2021;5:46 https://dx.doi.org/10.20517/2574-1209.2021.52
Figure 1. A 55-year-old male previously had bilateral partial inferior turbinectomy for persistent nasal obstruction without improvement
after surgery. (A) presents the left nasal vestibule with a visible collapse of the external nasal valve caused by nasal framework
disintegration. (B) presents beaded granular nasal mucosa throughout the entire nasal cavity. The patient was referred to a
rheumatologist and diagnosed with GPA.
Figure 2. A 39-year-old male patient with a left maxillary mass invading the left orbital contents. There is evident bony destruction with
osteoneogenesis. Endoscopic biopsy revealed necrotizing vascular inflammation with extensive necrosis. Red arrow: left nasal mass
extending intra-orbitally. Yellow arrow: bone destruction of the medial wall of the left maxilla and medial orbital wall.
including vasculitis, granuloma, and necrosis were found in only 16% of the patients, while only 21% had
vasculitis and granulomatous changes, and only 23% had vasculitis and necrosis changes [Figure 3]. Thus, a
negative biopsy does not rule out a diagnosis of ANCA vasculitis. However, a biopsy can still be of value in
ANCA-negative patients and where other diagnoses, such as malignancy and infection, need to be excluded.
In cases of generalized involvement, biopsies from other organs such as kidney and skin are more
sensitive [28,29] .
Treatment
A multidisciplinary approach for the management of cases of ANCA-associated vasculitides involving the
upper respiratory tract is highly recommended.
Medical treatment
If there is involvement of a major organ such as lung or kidney or there is life-threatening disease,
aggressive treatment should be initiated. For localized disease involving sinuses and the upper airway, a less
aggressive approach can be used. Methotrexate is recommended for induction of remission. Mycophenolate