OBJECTIVES ANCA-associated vasculitis (AAV) can affect all age groups. We aimed to show that differences in disease presentation and 6 month outcome between younger- and older-onset patients are still incompletely understood. METHODS We included patients enrolled in the Diagnostic and Classification Criteria for Primary Systemic Vasculitis (DCVAS) study between October 2010 and January 2017 with a diagnosis of AAV. We divided the population according to age at diagnosis: <65 years or ≥65 years. We adjusted associations for the type of AAV and the type of ANCA (anti-MPO, anti-PR3 or negative). RESULTS A total of 1338 patients with AAV were included: 66% had disease onset at <65 years of age [female 50%; mean age 48.4 years (s.d. 12.6)] and 34% had disease onset at ≥65 years [female 54%; mean age 73.6 years (s.d. 6)]. ANCA (MPO) positivity was more frequent in the older group (48% vs 27%; P = 0.001). Younger patients had higher rates of musculoskeletal, cutaneous and ENT manifestations compared with older patients. Systemic, neurologic,cardiovascular involvement and worsening renal function were more frequent in the older-onset group. Damage accrual, measured with the Vasculitis Damage Index (VDI), was significantly higher in older patients, 12% of whom had a 6 month VDI ≥5, compared with 7% of younger patients (P = 0.01). Older age was an independent risk factor for early death within 6 months from diagnosis [hazard ratio 2.06 (95% CI 1.07, 3.97); P = 0.03]. CONCLUSION Within 6 months of diagnosis of AAV, patients >65 years of age display a different pattern of organ involvement and an increased risk of significant damage and mortality compared with younger patients.

中文翻译：

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抗中性粒细胞胞浆抗体相关血管炎的发病年龄与临床表现和短期结果之间的关系

目的 ANCA 相关性血管炎 (AAV) 可影响所有年龄组。我们的目的是表明年轻患者和老年患者之间疾病表现和 6 个月结果的差异仍未完全了解。方法 我们纳入了 2010 年 10 月至 2017 年 1 月期间参加原发性系统性血管炎诊断和分类标准 (DCVAS) 研究且诊断为 AAV 的患者。我们根据诊断时的年龄划分人群：<65 岁或≥65 岁。我们调整了 AAV 类型和 ANCA 类型（抗 MPO、抗 PR3 或阴性）的关联。结果 共纳入 1338 名 AAV 患者：66% 的发病年龄小于 65 岁 [女性 50%；平均年龄 48.4 岁 (sd 12.6)]，34% 的患者发病年龄≥65 岁 [女性 54%；平均年龄 73.6 岁 (sd 6)]。ANCA (MPO) 阳性在老年组中更为常见（48% 对 27%；P = 0.001）。与老年患者相比，年轻患者的肌肉骨骼、皮肤和耳鼻喉表现的发生率更高。全身、神经、心血管受累和肾功能恶化在老年组中更为常见。使用血管炎损伤指数 (VDI) 衡量的损伤累积在老年患者中显着更高，其中 12% 的 6 个月 VDI ≥5，而年轻患者为 7% (P = 0.01)。年龄较大是诊断后 6 个月内过早死亡的独立危险因素 [风险比 2.06 (95% CI 1.07, 3.97)；P = 0.03]。结论 在诊断 AAV 的 6 个月内，患者 >