This study investigated the characteristics of hypertrophic pachymeningitis (HP) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), using information from a multicenter study in Japan. We 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. Of 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). GPA 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.

中文翻译：

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ANCA 相关性血管炎中的肥厚性硬脑膜炎：日本的一项横断面和多机构研究 (J-CANVAS)

本研究利用来自日本的一项多中心研究的信息，调查了抗中性粒细胞胞质抗体 (ANCA) 相关性血管炎 (AAV) 中肥厚性硬脑膜炎 (HP) 的特征。我们分析了 663 名亚洲 AAV 患者（总 AAV）的临床信息，其中包括 558 名新诊断 AAV 患者和 105 名复发 AAV 患者。比较有和没有HP的患者的临床结果。为了阐明 HP 发展的相关表现，还进行了多变量逻辑回归分析。在 AAV 患者（平均年龄 70.2 ± 13.5 岁）中，30 例（4.52%）发现 HP，其中 20 例（3.58%）新诊断 AAV 和 10 例（9.52%）复发 AAV。50% 的 HP 患者将肉芽肿性多血管炎 (GPA) 分类。在总 AAV 和新诊断的 AAV 中，HP 患者的 GPA 患病率显着高于无 HP 患者（p < 0.001）。在新诊断的 AAV 中，HP 患者的血清蛋白酶 3 (PR3)-ANCA 阳性率显着高于无 HP 患者（p = 0.030）。HP 患者有明显的耳鼻喉 (ENT)（优势比 [OR] 1.48, 95% 置信区间 [CI] 1.03–2.14, p = 0.033）和粘膜/眼部表现（OR 5.99, 95% CI 2.59–13.86, p < 0.0001) 总 AAV。此外，他们显着有传导性听力损失（OR 11.6, 95% CI 4.51–29.57, p < 0.0001）和突发性视力丧失（OR 20.9, 95% CI 5.24–85.03, p < 0.0001）。GPA 主要见于 HP 患者。此外，在新诊断的 AAV 中，HP 患者的 PR3-ANCA 阳性率显着高于非 HP 患者。耳部和眼部表现可能与 HP 发展有关。