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

Abstract

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.

Anti-neutrophil Cytoplasmic Antibody-Associated Vasculitis With Periaortitis That Developed After mRNA COVID-19 Vaccination

“…In addition to the aforementioned vasculitis, syphilis-associated aortitis and infective aortitis can cause periarteritis. However, opinions on the differentiation of ANCA-associated large-vessel diseases, including the interpretation of the disease spectrum, are divided [7]. In this case, serum IgG4 was normal; furthermore, blood culture and syphilis serum markers were negative.…”

Spectrum of Large and Medium Vessel Vasculitis in Adults: Primary Vasculitides, Arthritides, Connective Tissue, and Fibroinflammatory Diseases

“…MPA and GPA partially overlap in clinical presentation, but only the later has a tendency for destructive mass lesions [ 46 ]. Most manifestations of AAV are due to SVV, and MVV/LVV are very rare (approximately 1–5%) [ 38 – 40 , 41 ••, 42 ]. Cerebral MVV was occasionally described [ 43 ].…”

“…Epstein-Barr-virus, parvovirus, human immunodeficiency virus, Streptococcus, SARS-Cov2), consider causes of coronary aneurysms [ 10 , 31 •] • SARS-Cov2-induced multisystem inflammatory syndrome can result in a Kawasaki-like presentation in young adults [ 1 ••] ANCA-associated vasculitis Usually > 40–50 Y/A (rare in children, more common with increasing age); f ≈ m; incidence all subtypes (~ 25/10^6/y), EGPA ~ 1–4/10^6 [ 7 ] +/- aorta (incl. periaortitis), ICA [ 38 – 40 ] +/- TA (symptoms can be like GCA), muscle, coronary, cerebral, ocular, hepatic, mesenteric, gastric (splanchnic vessel rarely with aneurysms and rupture) [ 41 ••– 44 ] MPA/GPA/EGPA: constitutional symptoms, arthralgia, lung fibrosis or hemorrhage, GN, purpura, neuropathy, peri-myocarditis, bowel ischemia/ulcers, polychondritis, digital gangrene, meningitis, stroke, scleritis, retinitis, keratitis, rhinosinusitis, sialadenitis. GPA: mass lesions, nasal septal perforation, otitis, subglottic stenosis, pachymeningitis.…”

Eosinophilic granulomatosis polyangiitis (EGPA) complicated with periaortitis, precipitating role of dupilumab? A case report a review of the literature

“… 1 Large vessels involvement, including periaortitis, has been described in AAVs, mainly in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). 2 3 With regard to EGPA, only four cases of periaortitis have been reported in the literature. 4–7 …”