Ryder Hilary F, Tafe Laura J, Burns Christopher M
Department of Internal Medicine, Dartmouth-Hitchcock Medical Center, Lebanon, New Hampshire, USA.
J Clin Rheumatol. 2009 Sep;15(6):295-9. doi: 10.1097/RHU.0b013e3181b1246d.
Aortitis is typically a chronic, progressive disease manifestation associated with large vessel vasculitidies, most notably giant cell, Takayasu arteritis, and a newly described entity, isolated aortitis. The aortitis may lead to aneurysm formation and symptoms associated with branch vessel occlusion in these diseases, but aortic dissection is rare and usually a late complication of smoldering, incompletely treated disease. We present a case of aortitis in a previously healthy 39-year-old man who succumbed to aortic dissection hours after the onset of symptoms. No aneurysm or fibrosis was found on postmortem examination. The inflammation was characterized by disruption of the media with patchy transmural chronic and focally acute inflammatory infiltrate. We review case reports of other individuals with aortitis, who initially or very early in their course presented with aortic dissection in the absence of known rheumatic disease and most without evidence of aneurysm formation. We believe that this represents a process characterized by an aggressive vasculitis of the aorta with its own clinical features, a fulminant variety of isolated aortitis.
主动脉炎通常是一种与大血管血管炎相关的慢性、进行性疾病表现，最显著的是巨细胞动脉炎、高安动脉炎，以及一种新描述的实体——孤立性主动脉炎。在这些疾病中，主动脉炎可能导致动脉瘤形成以及与分支血管闭塞相关的症状，但主动脉夹层很少见，通常是隐匿性、未得到充分治疗疾病的晚期并发症。我们报告一例主动脉炎病例，患者为一名39岁此前健康的男性，在症状出现数小时后死于主动脉夹层。尸检未发现动脉瘤或纤维化。炎症的特征是中膜破坏，伴有斑片状透壁慢性炎症和局灶性急性炎症浸润。我们回顾了其他主动脉炎患者的病例报告，这些患者在病程最初或非常早期就出现了主动脉夹层，且无已知的风湿性疾病，大多数也没有动脉瘤形成的证据。我们认为这代表了一种以主动脉侵袭性血管炎为特征的过程，具有其自身的临床特征，是一种暴发性的孤立性主动脉炎。
