PDF(Pubmed)
Abstract：
Behçet\'s disease is a chronic inflammatory condition that predominantly affects the body\'s blood vessels, exhibiting various clinical manifestations and complications. The exact cause remains unclear, but genetic predisposition, immune responses, and vascular activation are believed to contribute to its development. This disease is more prevalent in certain geographic regions and primarily affects young adults, particularly males. Pulmonary aneurysm, a complication of Behçet\'s disease, is the leading cause of mortality in Behcet disease. In this review, we summarize the complications of Behcet disease with a focus on pulmonary artery aneurysms. We discussed the medical, endovascular, and surgical management of pulmonary aneurysms in Behcet disease and the indications and outcomes of the different treatment options. Corticosteroids and cyclophosphamide remain the preferred first-line therapy. However, clinical improvement with infliximab or adalimumab, tumor necrosis factor-alpha (TNFα) blocking agents, have been reported after treatment failure with recommended first-line agents. In patients who fail medical therapy or those with life-threatening hemoptysis, endovascular or surgical intervention is the next option. Endovascular interventions include pulmonary artery embolization with coils or acrylic glue and using plugs, occluders, or stents. Endovascular interventions usually have fewer adverse effects than surgery. Although the risk of surgical procedures is high in pulmonary artery aneurysms, it could be a life-saving procedure in patients with life-threatening hemoptysis. Surgical options, including pulmonary artery ligation, aneurysmorrhaphy, segmentectomy, lobectomy, or pneumonectomy are available. However, the results of surgical therapy for Behçet aneurysms are often disappointing.
摘要：
Behçet病是一种主要影响人体血管的慢性炎症，表现出各种临床表现和并发症。确切原因尚不清楚,但是遗传倾向，免疫反应，和血管活化被认为有助于其发展。这种疾病在某些地理区域更为普遍，主要影响年轻人，尤其是男性。肺动脉瘤,Behçet病的并发症,是导致白塞病死亡的主要原因.在这次审查中,我们总结了Behcet病的并发症，重点是肺动脉动脉瘤。我们讨论了医学，血管内,白塞病肺动脉瘤的外科治疗以及不同治疗方案的适应症和结果。皮质类固醇和环磷酰胺仍然是首选的一线治疗。然而,英夫利昔单抗或阿达木单抗的临床改善，肿瘤坏死因子-α（TNFα）阻断剂，在推荐的一线药物治疗失败后报告。在药物治疗失败或危及生命的咯血患者中，血管内或手术干预是下一个选择.血管内介入治疗包括用线圈或丙烯酸胶栓塞肺动脉并使用塞子，封堵器,或支架。血管内介入治疗通常比手术有更少的不良反应。尽管肺动脉动脉瘤的手术风险很高，这可能是危及生命的咯血患者的救命程序。手术选择,包括肺动脉结扎,动脉瘤缝合术,节段切除术,肺叶切除术,或肺切除术是可用的。然而,Behçet动脉瘤的手术治疗结果往往令人失望.
