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Page 2 of 3 Quartuccio. Vessel Plus 2022;6:23 https://dx.doi.org/10.20517/2574-1209.2021.127

The clinical presentation can be quite varied, ranging from skin vasculitis to fulminant multisystem disease.
Typical features of GPA include nasal crusting, epistaxis, uveitis, orbital mass, upper respiratory tract
involvement with bronchial or tracheal stenosis, and often renal involvement. Patients with MPA present
with more severe renal disease than GPA, together with polyneuropathy. EGPA typically presents with a
multisystem disease (involving in particular the kidneys, heart, and peripheral nerves) on a background of
[5]
asthma of adult onset, nasal polyposis, and peripheral blood or tissue eosinophilia .
In the large majority of patients, an induction of remission using cyclophosphamide or rituximab should be
considered at the onset of AAV. Other induction regimens with methotrexate or mycophenolate mofetil
should only be considered for patients with limited disease, without major organ involvement or life-
threatening disease [6]

Pathogenesis of AAV involves B and T lymphocytes, endothelial cells, monocytes, ANCAs, and the
alternative complement pathway .
[7]

Rituximab was approved by the Food and Drug Administration (FDA) in the 2011 in the induction
treatment of patients with GPA and MPA, after two randomized trials demonstrated non-inferiority of
rituximab to cyclophosphamide in inducing remission in both new and relapsed patients with GPA and
MPA . Rituximab is also recommended in maintaining remission in patients with GPA and MPA .
[5]
[5]
The optimal duration in terms of both efficacy and safety of rituximab maintenance remains still unknown,
and optimizing B cell-depleting therapy as well as minimizing glucocorticoid regimens are main topics in
[8]
the research agenda of AAV .
Very recently (October 8, 2021), avacopan, an orally administered selective complement 5a receptor
inhibitor, has been approved by FDA as an adjunctive treatment of adult patients with severe active AAV, in
[9]
combination with standard therapy . It may represent a new step towards steroid-free regimens in AAV.
A better clinical and molecular characterization of AAV should drive this new drug discovery era in AAV in
[10]
order to reach the ambitious goal of precision medicine in AAV .

DECLARATIONS
Authors’ contribution
The author contributed solely to the article.

Availability of data and materials
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Financial support and sponsorship
None.

Conflicts of interest
The author declared that there are no conflicts of interest.

Ethical approval and consent to participate
Not applicable.