Hughes-Stovin syndrome (HSS) is a rare vasculitis of unknown etiology. The disease is characterized by pronounced inflammation and damage to the vessel walls, with subsequent widespread vascular thrombosis and the formation of pulmonary artery aneurysms that can lead to fatal hemoptysis. This disorder can be mistaken for other conditions, such as chronic thromboembolic pulmonary disease (CTEPD) without or with pulmonary hypertension at rest (CTEPH).

We report the case of a 20-year-old female with HSS, which was misdiagnosed as CTEPH and subsequently treated with anticoagulants, which led to severe hemoptysis and eventually death of the patient. This case highlights the challenges of diagnosing HSS at early stages of the disease.

HSS should be considered in young patients with signs of large vessel vasculitis in combination with thrombotic occlusions of pulmonary arteries, with or without aneurysms of the pulmonary arteries, and particularly, if there are no risk factors for thromboembolic disease.

休斯-斯托文综合征 (HSS) 是一种病因不明的罕见血管炎。该疾病的特点是明显的炎症和血管壁损伤，随后出现广泛的血管血栓和肺动脉瘤的形成，可导致致命的咯血。这种疾病可能会被误认为是其他疾病，例如慢性血栓栓塞性肺病 (CTEPD)，不伴有或伴有静息时肺动脉高压 (CTEPH)。

我们报告一例20岁女性HSS患者，被误诊为CTEPH，随后接受抗凝药物治疗，导致患者严重咯血，最终死亡。该病例凸显了在疾病早期诊断 HSS 的挑战。

对于有大血管血管炎症状并伴有肺动脉血栓性闭塞的年轻患者，无论有或没有肺动脉瘤，尤其是在没有血栓栓塞性疾病危险因素的情况下，应考虑HSS。