Abstract：
BACKGROUND: Aortitis is an important form of vasculitis with significant risk of complications. Very few studies have provided detailed clinical phenotyping across the whole disease spectrum. Our primary aim was to look the clinical features, management strategies and complications associated with non-infectious aortitis.
METHODS: A retrospective review was performed on patients with diagnosis of noninfectious aortitis at the Oxford University hospitals NHS Foundation Trust. Clinicopathologic features were recorded including demographics, presentation, aetiology, laboratory, imaging findings, histopathology, complications, treatment, and outcome.
RESULTS: We report the data on 120 patients (59% females). Systemic inflammatory response syndrome constituted the most common presentation (47.5%). 10.8% were diagnosed following a vascular complication (dissection or aneurysm). All patients (n = 120) had raised inflammatory markers (median ESR 70.0 mm/h and CRP 68.0 mg/L). Isolated aortitis subgroup (15%) had significantly higher likelihood of presenting with vascular complications and challenging to diagnose due to non-specific symptoms. Prednisolone (91.5%) and methotrexate (89.8%) were the most used treatment. 48.3% developed vascular complications during the disease course including ischaemic complications (25%), aortic dilatation and aneurysms (29.2%) and dissection (4.2%). Risk of dissection was higher in the isolated aortitis subgroup at 16.6% compared to all other types of aortitis at 1.96%.
CONCLUSIONS: Risk of vascular complications is high in non-infectious aortitis patients during disease course, hence early diagnosis and appropriate management is key. DMARDs such as Methotrexate appear to be effective, nonetheless there remain gaps in evidence for longer-term management of relapsing disease. Dissection risk seems much higher for patients with isolated aortitis.
METHODS: A retrospective review was performed on patients with diagnosis of noninfectious aortitis at the Oxford University hospitals NHS Foundation Trust. Clinicopathologic features were recorded including demographics, presentation, aetiology, laboratory, imaging findings, histopathology, complications, treatment, and outcome.
RESULTS: We report the data on 120 patients (59% females). Systemic inflammatory response syndrome constituted the most common presentation (47.5%). 10.8% were diagnosed following a vascular complication (dissection or aneurysm). All patients (n = 120) had raised inflammatory markers (median ESR 70.0 mm/h and CRP 68.0 mg/L). Isolated aortitis subgroup (15%) had significantly higher likelihood of presenting with vascular complications and challenging to diagnose due to non-specific symptoms. Prednisolone (91.5%) and methotrexate (89.8%) were the most used treatment. 48.3% developed vascular complications during the disease course including ischaemic complications (25%), aortic dilatation and aneurysms (29.2%) and dissection (4.2%). Risk of dissection was higher in the isolated aortitis subgroup at 16.6% compared to all other types of aortitis at 1.96%.
CONCLUSIONS: Risk of vascular complications is high in non-infectious aortitis patients during disease course, hence early diagnosis and appropriate management is key. DMARDs such as Methotrexate appear to be effective, nonetheless there remain gaps in evidence for longer-term management of relapsing disease. Dissection risk seems much higher for patients with isolated aortitis.
摘要：
背景：主动脉炎是血管炎的一种重要形式，具有严重的并发症风险。很少有研究提供了整个疾病谱的详细临床表型。我们的主要目的是观察临床特征，与非感染性主动脉炎相关的管理策略和并发症。
方法：对牛津大学NHSFoundationTrust医院诊断为非感染性主动脉炎的患者进行了回顾性研究。记录临床病理特征，包括人口统计学，介绍,病因学，实验室,影像学发现，组织病理学,并发症,治疗,和结果。
结果：我们报告了120例患者（59%为女性）的数据。全身炎症反应综合征是最常见的表现(47.5%)。10.8％被诊断为血管并发症（夹层或动脉瘤）。所有患者（n=120）的炎症标志物升高（ESR中位数为70.0mm/h，CRP为68.0mg/L）。孤立性主动脉炎亚组（15％）出现血管并发症的可能性明显更高，并且由于非特异性症状而难以诊断。泼尼松龙（91.5％）和甲氨蝶呤（89.8％）是最常用的治疗方法。48.3%的人在疾病过程中出现血管并发症，包括缺血并发症(25%)，主动脉扩张和动脉瘤（29.2％）和夹层（4.2％）。孤立性主动脉炎亚组的夹层风险较高，为16.6％，而所有其他类型的主动脉炎为1.96％。
结论：非感染性主动脉炎患者在病程中发生血管并发症的风险较高，因此早期诊断和适当管理是关键.甲氨蝶呤等DMARD似乎有效，尽管如此,对于复发性疾病的长期治疗仍存在证据空白.孤立性主动脉炎患者的夹层风险似乎更高。
方法：对牛津大学NHSFoundationTrust医院诊断为非感染性主动脉炎的患者进行了回顾性研究。记录临床病理特征，包括人口统计学，介绍,病因学，实验室,影像学发现，组织病理学,并发症,治疗,和结果。
结果：我们报告了120例患者（59%为女性）的数据。全身炎症反应综合征是最常见的表现(47.5%)。10.8％被诊断为血管并发症（夹层或动脉瘤）。所有患者（n=120）的炎症标志物升高（ESR中位数为70.0mm/h，CRP为68.0mg/L）。孤立性主动脉炎亚组（15％）出现血管并发症的可能性明显更高，并且由于非特异性症状而难以诊断。泼尼松龙（91.5％）和甲氨蝶呤（89.8％）是最常用的治疗方法。48.3%的人在疾病过程中出现血管并发症，包括缺血并发症(25%)，主动脉扩张和动脉瘤（29.2％）和夹层（4.2％）。孤立性主动脉炎亚组的夹层风险较高，为16.6％，而所有其他类型的主动脉炎为1.96％。
结论：非感染性主动脉炎患者在病程中发生血管并发症的风险较高，因此早期诊断和适当管理是关键.甲氨蝶呤等DMARD似乎有效，尽管如此,对于复发性疾病的长期治疗仍存在证据空白.孤立性主动脉炎患者的夹层风险似乎更高。
