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[抗中性粒细胞胞浆抗体相关血管炎中的大血管受累:一例报告]

[Large vessel involvement in ANCA-associated vasculitis: Report of one case].

作者信息

Vega Jorge, Guarda Francisco J

出版信息

Rev Med Chil. 2015 Sep;143(9):1206-9. doi: 10.4067/S0034-98872015000900015.

Abstract

Pauci-immune glomerulonephritis in systemic vasculitides usually have anti-neuthrophil cytoplasmic antibodies (ANCA). However, vasculitides of large vessels such as Takayasu's and giant cell (temporal) arteritis do not. Exceptionally ANCA(+) small vessel vasculitides are associated with large vessel vasculitis. It may be a coincidence or both vasculitides have a common pathogenesis. We report a 30 years old woman on hemodialysis due to a chronic glomerulonephritis ANCA(+) diagnosed nine years ago. Eight years later, she presented with an aortitis with severe stenosis of distal aorta and vasculitis of left subclavian artery. She was treated with adrenal steroids and cyclophosphamide. During the ensuing five years she has been stable and without signs of reactivation of the disease.

摘要

系统性血管炎中的寡免疫性肾小球肾炎通常存在抗中性粒细胞胞浆抗体(ANCA)。然而,大动脉血管炎如高安氏病和巨细胞(颞)动脉炎则不然。极少数情况下,ANCA阳性的小血管血管炎与大血管血管炎相关。这可能是巧合,也可能两种血管炎有共同的发病机制。我们报告一名30岁女性,因九年前诊断为慢性肾小球肾炎ANCA阳性而接受血液透析。八年后,她出现主动脉炎,伴有远端主动脉严重狭窄和左锁骨下动脉血管炎。她接受了肾上腺皮质类固醇和环磷酰胺治疗。在随后的五年里,她病情稳定,没有疾病复发的迹象。

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