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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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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
PubMed
Summary
This summary is machine-generated.

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.

Keywords:
ANCA associated vasculitisAortaLarge vessel involvementNeurological involvement

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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.