Life-Threatening Anticoagulation in Hughes-Stovin Syndrome with Pulmonary Arterial Thrombosis: A Case Report

Hughes-Stovin syndrome (HSS) is a rare variant of Behçet’s disease characterized by the presence of pulmonary vascular aneurysms and venous thrombosis. In this case report, our aim is to emphasize the response to medical treatment in a patient currently under our follow-up, whose general condition is progressing well. A 35-year-old male patient with a diagnosis of HSS presented to the emergency department with complaints of chest pain, dyspnea, and hemoptysis. Clinical, laboratory, and radiological findings were consistent with a diagnosis of massive pulmonary arterial thromboembolism. Pulmonary artery pressure (PAP) was measured at 95 mmHg during right heart catheterization. Chronic thrombi were observed in bilateral lower extremity Doppler ultrasonography. Despite the dilemma of anticoagulant treatment due to the presence of aneurysms in the pulmonary artery and the risk of bleeding associated with HSS, intensive care unit administration of heparin infusion was initiated due to the patient’s life-threatening massive pulmonary thromboembolism. With the improvement of vital signs, the patient was transferred to the chest diseases service, and treatment with low molecular weight heparin (enoxaparin sodium) was initiated. Furthermore, based on a multidisciplinary council decision, infliximab (intravenous, 5 mg/kg) and riociguat (oral, 2.5 mg three times a day) treatments were planned due to chronic thromboembolic pulmonary hypertension. At the 1-month follow-up after discharge, a decrease in PAP to 65 mmHg was observed, along with improvements in exercise capacity, functional class, and hemodynamics. HSS is a clinical condition with a poor prognostic course and high mortality rates, which can manifest with thrombosis and cardiovascular problems. Treatment consensus remains elusive in this context. Anticoagulants, antithrombotics, and immunosuppressive drugs are recommended. In cases where surgical treatment is not feasible, medical treatment can be a suitable option for clinicians. Particularly, for HSS cases unresponsive to corticosteroids, biological agents should be considered.