主动脉炎是一种罕见的疾病实体,患病率未知。原发性主动脉炎主要累及胸主动脉。最常通过PET对主动脉壁的III级18-FDG摄取进行成像诊断。或通过CT或MRI上的周向增厚>2.2mm,并进行后期对比。很少,经组织学证实的主动脉炎,如在计划的主动脉瘤手术后或主动脉夹层手术中发现的一些临床孤立性主动脉炎病例。最常见的组织学类型是肉芽肿/巨细胞或淋巴浆细胞。与主动脉炎相关的临床体征通常是非特异性的:虚弱,发烧,干咳,胸部,回来,腰部或腹部疼痛。主动脉炎可分为不同的病因类别:原发性主动脉炎,其中包括对主动脉壁具有优先或排他性向性的血管炎,继发于全身性或医源性疾病的主动脉炎,和感染性主动脉炎.原发性主动脉炎的主要病因是巨细胞动脉炎(GCA),大动脉炎(TA)或临床孤立性主动脉炎。继发于全身性疾病的主动脉炎见于萎缩型多软骨炎,系统性狼疮和炎症性风湿性疾病,如脊柱关节病和类风湿性关节炎。在ACG和AT中,主动脉炎是一个负面因素,以复发风险较高为特征,心血管并发症和死亡率增加。主动脉炎的管理不够规范,依赖于对心血管危险因素的控制,特别监测血压和低密度脂蛋白胆固醇,以及皮质类固醇治疗和免疫抑制药物,其使用将取决于与主动脉炎相关的疾病,最初的严重程度和合并症。
Aortitis is a rare disease entity of unknown prevalence. Primary
aortitis mainly affects the thoracic aorta. They are most often diagnosed on imaging by grade III 18-FDG uptake of the aortic wall on PET, or by circumferential thickening>2.2mm on CT or MRI with late-stage contrast. More rarely,
aortitis is histologically proven, as in some cases of clinically isolated
aortitis discovered after planned aortic aneurysm surgery or during aortic dissection surgery. The most common histological types are granulomatous/giant cell or lymphoplasmacytic. Clinical signs associated with aortitis are often non-specific: asthenia, fever, dry cough, chest, back, lumbar or abdominal pain.
Aortitis can be divided into different etiological categories: primary
aortitis, which includes vasculitis with a preferential or exclusive tropism for the aortic wall, aortitis secondary to systemic or iatrogenic diseases, and infectious
aortitis. The main etiologies of primary aortitis are giant cell arteritis (GCA), Takayasu arteritis (TA) or clinically isolated
aortitis. Aortitis secondary to systemic diseases is seen in atrophying polychondritis, systemic lupus and inflammatory rheumatic diseases such as spondyloarthropathy and rheumatoid arthritis. In both ACG and AT, aortitis is a negative factor, characterized by a higher risk of relapse, cardiovascular complications and increased mortality. The management of aortitis is insufficiently codified, and relies on the control of cardiovascular risk factors, with particular monitoring of blood pressure and LDL cholesterol, and on corticosteroid therapy and immunosuppressive drugs, the use of which will depend on the disease associated with the aortitis, the initial severity and comorbidities.