Pipitone N, Salvarani C, Peter H H
Rheumatologische Abteilung und Vaskulitis-Zentrum, Arcispedale S. M. Nuova, Reggio Emilia, Emilia, Italien.
Internist (Berl). 2010 Jan;51(1):45-52. doi: 10.1007/s00108-009-2407-x.
Chronic periaortitis is a rare fibroinflammatory disorder which affects the abdominal aorta and may spread into the retroperitoneum, often encasing the ureters. An aneurysma of the abdominal aorta and vasculitis of the thoracic aorta and of supra-aortic vessels may also coexist. Chronic periaortitis can be idiopathic or secondary to different triggers such as drugs, tumors and infections. Abdominal and/or low back pain is the hallmark symptom. Laboratory markers of inflammation are usually increased. The diagnosis rests on computerized tomography or magnetic resonance imaging, which typically show a retroperitoneal mass displacing the aorta anteriorly and the ureters medially. Positron-emission tomography may assist in defining disease activity and extension. Chronic periaortitis should be differentiated from other fibrosing disorders of various origins. Histology is required in atypical cases to secure the diagnosis. Treatment is based on high-dose steroids with a tapering scheme combined with immunosuppressive agents in refractory or relapsing disease. In case of ureter obstruction early DJ-catheter placement is required. Operative interventions to relieve ureter obstruction are rarely necessary provided immunosuppressive treatment is timely instituted.
慢性主动脉周炎是一种罕见的纤维炎症性疾病,累及腹主动脉,可蔓延至腹膜后间隙,常包绕输尿管。腹主动脉瘤以及胸主动脉和主动脉弓上血管的血管炎也可能并存。慢性主动脉周炎可为特发性,或继发于药物、肿瘤和感染等不同诱因。腹部和/或下背部疼痛是其标志性症状。炎症的实验室指标通常升高。诊断依靠计算机断层扫描或磁共振成像,其典型表现为腹膜后肿块使主动脉向前移位,输尿管向内侧移位。正电子发射断层扫描有助于明确疾病活动度和范围。慢性主动脉周炎应与其他各种原因引起的纤维化疾病相鉴别。非典型病例需要组织学检查以确诊。治疗基于大剂量类固醇并采用逐渐减量方案,难治性或复发性疾病需联合免疫抑制剂。若出现输尿管梗阻,需早期置入DJ导管。只要及时进行免疫抑制治疗,很少需要手术干预来缓解输尿管梗阻。