ArticleArthritis research & therapy2022
Hypertrophic pachymeningitis in ANCA-associated vasculitis: a cross-sectional and multi-institutional study in Japan (J-CANVAS).
Article in Arthritis research & therapy, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers.
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9 citing papers in PubMed, 18 citations in OpenAlex.
- ANCA-associated vasculitis with thoracic spinal canal dural involvement and hypertrophic cranial pachymeningitis: A case report.Medicine · 2026Article
- Quantitative assessment of intrapulmonary vessel volume by CTPA in vasculitis patients with pulmonary vascular involvement.BMC medical imaging · 2026Article
- IgG4-related disease and other fibro-inflammatory conditions.Nature reviews. Rheumatology · 2025Review
- Myelopathy Due to Hypertrophic Pachymeningitis Associated With ANCA Vasculitis: A 10-Year Follow-up Case Report.Korean journal of neurotrauma · 2024Article
- Two cases of MPO-ANCA-positive hypertrophic pachymeningitis mimicking as intracranial infection.BMC neurology · 2024Article
- Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment.Rheumatology international · 2024Review
- Clinical characteristics of hypertrophic cranial pachymeningitis in granulomatosis with polyangiitis: a retrospective single-center study in China.Arthritis research & therapy · 2024Article
- Rituximab for treatment of pachymeningitis in granulomatosis with polyangiitis.BMJ case reports · 2023Article
- Spinal Hypertrophic Pachymeningitis in Antineutrophilic Cytoplasmic Antibody-Negative Vasculitis: A Case Report.Cureus · 2023Article
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34 authors at 20 institutions in 1 country.
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Abstract
backgroundThis study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan.
methodsWe analyzed the clinical information of 663 Asian patients with AAV (total AAV), including 558 patients with newly diagnosed AAV and 105 with relapsed AAV. Clinical findings were compared between patients with and without HP. To elucidate the relevant manifestations for HP development, multivariable logistic regression analyses were additionally performed.
resultsOf the patients with AAV (mean age, 70.2 ± 13.5 years), HP was noted in 30 (4.52%), including 20 (3.58%) with newly diagnosed AAV and 10 (9.52%) with relapsed AAV. Granulomatosis with polyangiitis (GPA) was classified in 50% of patients with HP. A higher prevalence of GPA was significantly observed in patients with HP than in those without HP in total AAV and newly diagnosed AAV (p < 0.001). In newly diagnosed AAV, serum proteinase 3 (PR3)-ANCA positivity was significantly higher in patients with HP than in those without HP (p = 0.030). Patients with HP significantly had ear, nose, and throat (ENT) (odds ratio [OR] 1.48, 95% confidence interval [CI] 1.03-2.14, p = 0.033) and mucous membrane/eye manifestations (OR 5.99, 95% CI 2.59-13.86, p < 0.0001) in total AAV. Moreover, they significantly had conductive hearing loss (OR 11.6, 95% CI 4.51-29.57, p < 0.0001) and sudden visual loss (OR 20.9, 95% CI 5.24-85.03, p < 0.0001).
conclusionGPA was predominantly observed in patients with HP. Furthermore, in newly diagnosed AAV, patients with HP showed significantly higher PR3-ANCA positivity than those without HP. The ear and eye manifestations may be implicated in HP development.
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