Qadir Abdul, Abdellatif Amal Wael, Waheed Aamir, Islam Mamunul
Department of Medicine, Hamad Medical Corporation HGH, Doha, Qatar.
Medical Education Department, Hamad Medical Corporation, Doha, Qatar.
Int J Rheum Dis. 2025 Apr;28(4):e70227. doi: 10.1111/1756-185X.70227.
Hughes-Stovin Syndrome (HSS) is a rare, life-threatening condition characterized by thrombophlebitis and pulmonary artery aneurysms, often viewed as a variant of Behçet's disease. Its diagnosis and management are challenging due to its rarity and overlapping symptoms with other vascular disorders. A 33-year-old male presented with massive hemoptysis and a history of recurrent oral and scrotal ulcers. Imaging revealed bilateral pulmonary emboli, a pulmonary artery aneurysm, and a large right atrial thrombus. These findings, coupled with his clinical history, led to a diagnosis of HSS. A multidisciplinary team initiated anticoagulation for the thrombus and immunosuppressive therapy (steroids, cyclophosphamide). Follow-up imaging showed improvement in both the aneurysm and thrombus. This case highlights the complexity of diagnosing and managing HSS. Early recognition, supported by a collaborative approach, is critical to improving outcomes in this rare syndrome.
休斯-斯托文综合征(HSS)是一种罕见的、危及生命的疾病,其特征为血栓性静脉炎和肺动脉瘤,常被视为白塞病的一种变体。由于其罕见性以及与其他血管疾病症状重叠,其诊断和治疗颇具挑战性。一名33岁男性出现大量咯血,并有复发性口腔和阴囊溃疡病史。影像学检查显示双侧肺栓塞、一个肺动脉瘤和一个巨大的右心房血栓。这些发现,结合他的临床病史,导致诊断为HSS。一个多学科团队开始对血栓进行抗凝治疗,并给予免疫抑制治疗(类固醇、环磷酰胺)。随访影像学检查显示动脉瘤和血栓均有改善。该病例突出了HSS诊断和治疗的复杂性。在协作方法的支持下尽早识别,对于改善这种罕见综合征的治疗效果至关重要。