Large-vessel involvement in ANCA-associated vasculitis: A multicenter case-control study
V Monghal1, X Puéchal2, P Smets1
1Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France.
Seminars in Arthritis and Rheumatism
|May 29, 2024
Summary
Large-vessel involvement (LVI) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is rare but associated with neurological issues. Standard AAV treatments are effective for managing LVI.
Area of Science:
- Rheumatology
- Vascular Medicine
- Immunology
Background:
- Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) typically affects small vessels.
- Large-vessel involvement (LVI) is an uncommon but significant manifestation of AAV.
Purpose of the Study:
- To characterize LVI in AAV patients.
- To identify risk factors associated with LVI.
- To describe the therapeutic management of LVI.
Main Methods:
- A multicenter case-control study (1:2 ratio) was conducted.
- Patients met ACR/EULAR classification criteria for AAV and Chapel Hill nomenclature for LVI.
- Controls were matched for age, sex, and AAV type.
Main Results:
- Twenty-six AAV patients with LVI were included, predominantly with granulomatosis with polyangiitis.
- The aorta was the most frequently affected vessel (69%), followed by supra-aortic trunks (35%).
- LVI was significantly associated with neurological manifestations (OR=3.23) but not cardiovascular risk factors or AAV relapse.
Conclusions:
- LVI should be considered a potential manifestation of AAV, with the aorta being commonly affected.
- Neurological involvement in AAV may increase the risk of developing LVI.
- Standard treatments for AAV, including corticosteroids and immunosuppressants (cyclophosphamide, rituximab), are effective for managing LVI.
