Scientific article
Open access

Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study

Published inRheumatology international, vol. 41, no. 12, p. 2147-2156
Publication date2021-12
First online date2021-09-24

ANCA-associated vasculitis (AAV) in general involves small blood vessels and includes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Although reported in a few studies, the prevalence of large vessel vasculitis (LVV) in patients with AAV remains to be further explored. The goal of the present study was to assess the prevalence of LVV in a cohort of patients with AAV and to characterize this population. We conducted a ten-year retrospective study of a single-center cohort of AAV, including 101 patients with GPA (n = 58), EGPA (n = 28), MPA (n = 15), and compared the groups with or without associated LVV. LVV was diagnosed in five patients, two with aortitis and three with temporal arteritis, corresponding to a total prevalence of 5.0% [95% CI 1.6–11.2%]. This value was significantly higher than the estimated prevalence of LVV in the normal Swiss population (OR 234.9 95% CI 91.18–605.2, p < 0.001). All five patients had GPA, whereas no cases with EGPA or MPA were identified. Anti-PR3 antibodies were detected in four out of five patients, anti-MPO in one patient. Since LVV can occur in a significant proportion of patients with GPA, evaluation for LVV may be considered systematically in the diagnostic workup of AAV.

  • ANCA-associated vasculitis
  • Antineutrophil cytoplasmic antibodies
  • Aortitis
  • Eosinophilic granulomatosis with polyangiitis
  • Granulomatosis with polyangiitis
  • Large vessel vasculitis
  • Microscopic polyangiitis
  • Temporal arteritis
Citation (ISO format)
COATTRENEC, Yann et al. Prevalence of large vessel vasculitis in ANCA-associated vasculitis: a retrospective cohort study. In: Rheumatology international, 2021, vol. 41, n° 12, p. 2147–2156. doi: 10.1007/s00296-021-04993-2
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Article (Published version)
ISSN of the journal0172-8172

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Creation01/19/2022 10:28:00 AM
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