Department of Cardiovascular Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan.
Ann Thorac Surg. 2009 Dec;88(6):2031-4. doi: 10.1016/j.athoracsur.2009.04.125.
Aortic aneurysm caused by microscopic polyangiitis is very rare. It typically affects the small vessels. We report the case of a 36-year-old woman who was diagnosed with microscopic polyangiitis as a 26-year-old and who was treated with oral hydrocortisone for 10 years, and had an ascending aortic aneurysm and severe aortic insufficiency without dilatation of the sinus of Valsalva. Laboratory analysis showed high titers of serum perinuclear anti-neutrophil cytoplasmic antibodies. She underwent replacement of the ascending aorta and aortic valve on September 21, 2006. Pathologic analysis of the aneurysmal wall revealed fibrous thickening of the adventitia and intima with severe destruction and atrophy of the media. We believe that this is the first report in the literature of a surgical case of an aortic aneurysm complicated with microscopic polyangiitis.
显微镜下多血管炎导致的主动脉瘤非常罕见。它通常影响小血管。我们报告了一例 36 岁的女性病例，她在 26 岁时被诊断为显微镜下多血管炎，接受了 10 年的口服氢可的松治疗，并且没有瓦氏窦扩张的升主动脉瘤和严重的主动脉瓣关闭不全。实验室分析显示血清核周抗中性粒细胞胞浆抗体滴度高。她于 2006 年 9 月 21 日接受了升主动脉和主动脉瓣置换术。动脉瘤壁的病理分析显示外膜和内膜的纤维性增厚，中膜严重破坏和萎缩。我们认为这是文献中首例手术治疗显微镜下多血管炎合并主动脉瘤的病例报告。
