复发性肺动脉瘤：白塞病谱系中的休斯-斯托文综合征
Recurrent Pulmonary Aneurysms: Hughes-Stovin Syndrome on the Spectrum of Behçet Disease.
作者信息
Bennji Sami M, du Preez Leonard, Griffith-Richards Stephanie, Smit Derrick P, Rigby Jonathan, Koegelenberg Coenraad F N, Irusen Elvis M, Allwood Brian W
机构信息
Division of Pulmonology, Department of Medicine, Tygerberg Academic Hospital/Stellenbosch University, Cape Town, South Africa.
Division of Cardiothoracic Surgery, Department of Surgery, Tygerberg Academic Hospital/Stellenbosch University, Cape Town, South Africa.
出版信息
Chest. 2017 Nov;152(5):e99-e103. doi: 10.1016/j.chest.2017.07.015.
In this report, we describe a male patient who presented with recurrent life-threatening hemoptysis due to the sequential formation of multiple pulmonary aneurysms. Both pulmonary artery coil embolization and right lower lobectomy were performed, with limited success. The patient experienced extensive bilateral femoral DVT extending into the inferior vena cava, with massive hemoptysis, fulfilling the diagnosis of Hughes-Stovin syndrome. A final diagnosis of Behçet disease was made following extensive investigation, and the patient responded well to prednisone 20 mg orally and azathioprine 100 mg orally.
在本报告中，我们描述了一名男性患者，该患者因多发性肺动脉瘤的相继形成而反复出现危及生命的咯血。先后进行了肺动脉线圈栓塞术和右下肺叶切除术，但效果有限。患者出现广泛的双侧股静脉深静脉血栓形成并延伸至下腔静脉，同时伴有大量咯血，符合Hughes-Stovin综合征的诊断。经过广泛检查后最终诊断为白塞病，该患者对口服泼尼松20毫克和口服硫唑嘌呤100毫克反应良好。
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