变应性肉芽肿性血管炎累及大中动脉六例报道并文献复习

目的 总结变应性肉芽肿性血管炎(CSS)合并大、中动脉病变的临床表现及治疗,以提高对CSS合并大、中动脉病变的认识.方法 回顾性分析诊断明确且合并大、中动脉病变的6例CSS患者.并进行文献复习.结果 CSS累及大、中动脉病变的类型有闭塞、狭窄、栓塞、夹层、假性动脉瘤、动脉炎及动脉周围炎等.累及的动脉可包括冠状动脉、视网膜中央动脉、肠系膜动脉、椎动脉、基底动脉、颈动脉、主动脉及四肢动脉等.CSS累及大、中动脉病变似乎与年龄、病程及抗中性粒细胞胞质抗体无关.早期积极应用激素加免疫抑制剂,绝大部分患者预后好,部分患者需要手术及抗凝治疗.CSS累及冠状动脉时病情凶险,死亡率高.结论 CSS可以累及大、中动脉,且累及的动脉十分广泛,动脉病变类型多样,临床上应予以重视.

Churg-Strauss syndrome involving large-and medium-sized arteries: report of six cases and review of literature

Objective To summarize the clinical manifestations and treatment of Churg-Strauss syndrome (CSS) involving large and medium-sized arteries. Method Six cases were investigated retrospec-tively and literature were reviewed. Results The pathological category of large-and medium-sized arteries involved in CSS included occlusion, stenosis, thrombesis, dissection, aneurysm, arteritis, and periarterifis. The involved arteries included coronary artery, central retinal artery, mesenteric artery, vertebral artery, basilar artery, carotid artery, aorta, and arteries of extremities. The large-and medium-sized arteries involvement in patients with CSS might not associated with age, duration of disease, and the titer of perinuclear-antineutrophil cytoplasmic antibodies. Treatment with corticosteroid and immunosuppressive agents combined with surgical interventions or anticoagulation if needed might improve the prognosis in the early stage. It was particularly hazardous for coronary involvement in patients with CSS. Conclusion CSS can involve many kinds of large-and medium-sized arteries. The pathological category of large and medium-sized arteries involved in CSS is diverse. More attention should be paid to large and medium-sized arteries involvement in patients with CSS.