Massive haemoptysis secondary to pulmonary artery aneurysms in Hughes–Stovin syndrome: a variant of Behçet’s disease managed with immunosuppression and endovascular embolisation

We report a rare and potentially life-threatening case of a young male with Behçet’s disease complicated by Hughes–Stovin syndrome who presented with massive haemoptysis secondary to multiple pulmonary artery aneurysms with associated thrombosis. The patient presented to the emergency department with a 20-day history of cough and fever, followed by acute worsening haemoptysis over 2 days, accompanied by haemodynamic instability. Initial CT pulmonary angiography demonstrated multiple bilateral saccular pulmonary artery aneurysms involving the descending and segmental branches, with partial and complete thrombosis. Transthoracic echocardiography revealed an associated right ventricular thrombus.

The patient required emergency airway protection, blood transfusion and vasopressor support. A multidisciplinary team involving pulmonology, rheumatology, cardiothoracic surgery and interventional radiology was engaged. High-dose systemic corticosteroids and immunosuppressive therapy were initiated for underlying vasculitis. Given the inaccessibility of the aneurysms for surgical repair, endovascular embolisation was recommended as definitive management. Despite transient relapses of haemoptysis during subsequent admissions, the patient was stabilised with aggressive immunosuppression and successfully underwent vascular intervention.

This case highlights the importance of early recognition of vascular Behçet’s disease and Hughes–Stovin syndrome as rare but critical causes of massive haemoptysis in young patients. Hughes–Stovin syndrome is a rare vasculitic disorder characterised by pulmonary artery aneurysms and thrombosis and is considered a cardiovascular variant of Behçet’s disease. Rupture of pulmonary artery aneurysms frequently presents with massive haemoptysis and is associated with high mortality. Prompt multidisciplinary management combining immunosuppressive therapy and endovascular intervention is essential to prevent fatal complications.