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Page 10 of 18 Padoan et al. Vessel Plus 2021;5:41 https://dx.doi.org/10.20517/2574-1209.2021.41

Table 2. Differential diagnoses of ear, nose and throat inflammatory, granulomatous and necrotizing lesions
Inflammatory autoimmune
Infections Malignancies Substances
diseases
Bacterial ANCA-associated vasculitis Nasal NK-T-cell lymphoma (midline Cocaine-induced midline destructive
Tuberculosis 1. Granulomatosis with polyangiits lethal granuloma) lesions (CIMDL)
Leprosy 2. Eosinophilic granulomatosis with
Syphilis polyangiits
Rhinoscleroma 3. Microscopic polyangiits
Actinomicosis
Fungal Other autoimmune diseases Levamisole-induced Vasculitis (LIV)
Zygomycosis 1. IgG4-related disease
Dermatacietes 2. Sarcoidosis
Rhinosporidiosis 3. Rheumatoid arthritis
Blastomycosis 4. Relapsing polychondritis
Histoplasmosis 5. Systemic lupus erythematosus
Sporotrichosis
Coccidioidomycosis
Protozoal Propylthiouracil-induced vascultis
Leishmaniasis

nasal passages. Nasal lubricants applied directly to the mucosa or emollients added to nasal saline washes
can help reduce dried nasal mucus and soften crusts, making them easily removable . Topical application
[81]
of antibiotics may be useful to eradicate Staphylococcus aureus from the nose.

Systemic treatment
The combination of corticosteroids and immunosuppressants is the mainstay of AAV treatment. Among
immunosuppressive agents, cyclophosphamide is the conventional induction treatment for systemic or
[82]
[83]
diffuse GPA , while methotrexate is an alternative for forms of GPA that are not life-threatening .
Induction treatment allows remission to be achieved in more than 80% of cases. For maintenance treatment,
azathioprine is the most widely used . In localized disease, in addition to methotrexate and trimethoprim,
[84]
sulfamethoxazole also appears to be effective, with a reduction in the relapse rate, mainly in ENT, possibly
for action against Staphylococcus aureus .
[85]
Rituximab (RTX), a chimeric human-mouse monoclonal antibody against CD20, is nowadays approved and
widely used in the treatment of AAV , with a good safety profile . RTX can be used in combination with
[86]
[82]
corticosteroids as a first-line treatment for severe AAVs, particularly when cyclophosphamide is not
recommended .
[87]
Regarding ENT manifestations, RTX is not usually recommended in patients without life- or organ-
[82]
threatening manifestations . However, RTX as rescue therapy has been administered in GPA with
refractory ENT manifestations. A recent case series reported a good response of refractory OMAAV after
[88]
RTX treatment .
In a large retrospective cohort of 59 AAV patients with orbital masses, RTX resulted highly effective
(remission rate: RTX 91% vs. cyclophosphamide 52%). However, in this study, all patients received
glucocorticoids without a standardized protocol, RTX was administered only in 19% of the patients, and
there was no subgroup analysis between patients treated with RTX and patients treated with other
[89]
immunosuppressive agents to assess if other features/treatments could have influenced the outcome . In a
previous case series of refractory GPA treated with RTX, Holle et al. reported a much lower
[90]
remission/improvement rate in patients with orbital masses (44.4%). Nevertheless, it is important to stress
that the retroorbital disease is not recommended to be treated with a mild immunosuppressive regimen
(e.g., methotrexate or mycophenolate) .
[82]