Department of Cardiothoracic Surgery, The Aga Khan University Hospital, Karachi, Pakistan.
Medical College, The Aga Khan University Hospital, Karachi, Pakistan.
Ann Thorac Surg. 2020 Nov;110(5):e355-e356. doi: 10.1016/j.athoracsur.2020.03.091. Epub 2020 May 3.
Approximately 40 cases of Hughes-Stovin syndrome have been recorded in the literature. The disease process exhibits an indefinite nature. It shares histopathologic and clinical features with Behçet disease, although there is no established association. The natural course of the disease is fatal in most cases. We report a case of a 31-year-old man with Hughes-Stovin syndrome who initially presented with occasional hemoptysis and a history of deep vein thrombosis. There was evidence of right-sided lower lobe pulmonary artery aneurysm that was managed with a lobectomy, after which he developed aneurysmal recurrence in the contralateral lung. Histopathologic analysis identified myxoid degeneration in the hilar region.
据文献记载，大约有 40 例休斯-斯托文综合征病例。该病的病程具有不定性，其组织病理学和临床表现与白塞病相似，但尚未建立明确的关联。在大多数情况下，该病的自然病程是致命的。我们报告了一例 31 岁男性休斯-斯托文综合征患者，最初表现为偶尔咯血和深静脉血栓形成病史。有右侧下叶肺动脉动脉瘤的证据，行肺叶切除术治疗，之后对侧肺部出现动脉瘤复发。组织病理学分析显示，肺门区域有黏液样变性。
