Schirmer J H, Both M, Moosig F
Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt und Universitätsklinikum Schleswig-Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland,
Internist (Berl). 2013 Dec;54(12):1419-20, 1422, 1424-6. doi: 10.1007/s00108-013-3297-5.
Chronic periaortitis is an inflammatory and fibrosing disease presenting as periaortal fibrosis and formation of aortic aneurysms which are mostly localized in the retroperitoneum and occasionally in the mediastinum. Inflammatory vasculitic involvement of large vessels is also possible. In addition to symptoms of systemic inflammation, mechanical complications also occur whereby obstruction of the ureter is the most frequent. The diagnosis is made by contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) and if the findings are atypical the diagnosis should be confirmed by biopsy. After exclusion of a secondary genesis, in which case therapy of the underlying illness would be necessary, idiopathic chronic periaortitis can be treated with steroids. In cases of refractory and relapsing courses the administration of further immunosuppressive medication can be necessary. Duration of therapy, dosage and indications for immunosuppressive medication are currently unclear and have to be defined in further randomized controlled trials with larger cohorts. If complications occur, interventional or operative treatment can be necessary; in cases of hydronephrosis the placement of double-J-stents is usually sufficient.
慢性主动脉周炎是一种炎症性和纤维化疾病,表现为主动脉周围纤维化和主动脉瘤形成,主动脉瘤大多位于腹膜后,偶尔位于纵隔。大血管也可能发生炎症性血管炎累及。除了全身炎症症状外,还会出现机械性并发症,其中输尿管梗阻最为常见。诊断通过增强计算机断层扫描(CT)或磁共振成像(MRI)进行,如果结果不典型,则应通过活检确诊。排除继发性病因(在这种情况下需要治疗基础疾病)后,特发性慢性主动脉周炎可用类固醇治疗。对于难治性和复发性病程,可能需要使用进一步的免疫抑制药物。目前免疫抑制药物的治疗持续时间、剂量和适应症尚不清楚,必须在更大队列的进一步随机对照试验中加以明确。如果发生并发症,可能需要进行介入或手术治疗;对于肾积水,通常放置双J支架就足够了。