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Academic Journal of Health Life-Threatening Anticoagulation in Hughes-Stovin Syndrome with Pulmonary Arterial Thrombosis: A Case Report [Acad J Health]
Acad J Health. 2023; 1(2): 53-57 | DOI: 10.14744/ajh.2023.92486

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

Serap Duru1, Merve Özdoğan Algın1, Busra Oral1, Fatma Yıldırım1, Ertuğrul Çağrı Bölek2, Alper Sarı2, Ebru Revan1, Saadet Demirtas Inci3, Bahadir Gultekin4, Koray Hekimoglu5, Umit Kervan6
1Department of Chest Disease, Ankara Etlik City Hospital, Ankara, Türkiye
2Department of Rheumatology, Ankara Etlik City Hospital, Ankara, Türkiye
3Department of Cardiology, Ankara Etlik City Hospital, Ankara, Türkiye
4Department of Cardiovascular Surgery, Ankara Etlik City Hospital, Ankara, Türkiye
5Department of Radiology, Ankara Etlik City Hospital, Ankara, Türkiye
6Department of Cardiovascular Surgery, Bilkent City Hospital, Ankara, Türkiye

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.

Keywords: Behçet’s disease, Hughes-Stovin syndrome, pulmonary thromboembolism

Serap Duru, Merve Özdoğan Algın, Busra Oral, Fatma Yıldırım, Ertuğrul Çağrı Bölek, Alper Sarı, Ebru Revan, Saadet Demirtas Inci, Bahadir Gultekin, Koray Hekimoglu, Umit Kervan. Life-Threatening Anticoagulation in Hughes-Stovin Syndrome with Pulmonary Arterial Thrombosis: A Case Report. Acad J Health. 2023; 1(2): 53-57

Corresponding Author: Serap Duru, Türkiye
Manuscript Language: English