Cole Adam, Mandava Anupa
Internal Medicine, SUNY Upstate Medical University, Syracuse, USA.
Cureus. 2022 Sep 1;14(9):e28672. doi: 10.7759/cureus.28672. eCollection 2022 Sep.
Hughes-Stovin syndrome (HSS) is a rare autoimmune disease that is considered to be a variant of Behçet's disease. It typically presents with multiple venous thrombi, thrombophlebitis, as well as pulmonary and bronchial aneurysms. The disease is typically associated with high morbidity and mortality in those diagnosed. This case discusses a 28-year-old male with HSS complicated by venous cardiac emboli and a mechanical valve on chronic anticoagulation. Despite medical and surgical management, the patient experienced continued progression of his pulmonary aneurysms and excessive bleeding, eventually requiring lung transplantation.
休斯-斯托文综合征(HSS)是一种罕见的自身免疫性疾病,被认为是白塞病的一种变体。它通常表现为多发性静脉血栓、血栓性静脉炎以及肺和支气管动脉瘤。该疾病在确诊患者中通常具有较高的发病率和死亡率。本病例讨论了一名28岁患有HSS的男性,并发静脉心脏栓塞,且因慢性抗凝而植入了机械瓣膜。尽管进行了药物和手术治疗,患者的肺动脉瘤仍持续进展,且出血过多,最终需要进行肺移植。