Aortic Dissection in c-ANCA-Associated Vasculitis: A Case Report and Literature Review

c-ANCA相关性血管炎并发主动脉夹层:病例报告及文献综述

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Abstract

Cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA)-associated vasculitis usually affects medium-sized and small vessels, with aortic involvement extremely rare. While vasculitis commonly leads to systemic inflammation, large vessel complications like aortitis or dissection are less well-documented. A male in his 50s with hypertension presented with sudden-onset, severe chest pain radiating to his back and shortness of breath. Pain slightly improved with opioids. Initial CT showed moderate thickening of the thoracic aorta, suggestive of vasculitis. High-dose steroids were started. Autoimmune tests confirmed elevated c-ANCA and anti-proteinase 3 antibodies. A follow-up CT angiography later revealed a Stanford type A aortic dissection extending from the thoracic to the abdominal aorta, involving the superior mesenteric artery and inferior mesenteric artery. The patient was urgently transferred for surgical intervention. Initial treatment with steroids was based on suspected vasculitis. The discovery of a Stanford type A aortic dissection required urgent surgical repair. Timely imaging was critical in identifying this severe complication, leading to rapid transfer for surgery. This case highlights the rare but serious possibility of aortic involvement in c-ANCA-associated vasculitis. It underscores the importance of serial imaging to detect and manage severe complications early, emphasizing adaptable treatment strategies for better outcomes.

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