Concurrent Pulmonary Artery Aneurysm, Pulmonary Thrombosis, and Intracardiac Thrombus in Behçet's Disease: A Case...

Behçet's disease is a systematic, inflammatory disorder affecting vessels of all sizes. It affects both venous and arterial systems. Vascular involvement carries a high risk of morbidity and mortality. Knowing that Behçet's disease is the most common vasculitides that causes pulmonary artery aneurysms, with a mortality rate of around 25%, makes early detection crucial. Thrombosis in Behçet's disease is mainly caused by an inflammatory process rather than a thrombophilic state, thus vascular thrombosis control is achieved with immunosuppressant medications rather than anticoagulants. An exception to the use of anticoagulants in Behçet's disease appears to be due to cerebral venous thrombosis. The occurrence of multiple site thrombosis and aneurysm simultaneously makes the management very challenging, as we will highlight in our case. We present a case of a 31-year-old female patient with many prior hospitalizations due to cerebral venous thrombosis, bilateral pulmonary thrombi, right ventricular thrombus, and right pulmonary artery aneurysm. The patient was diagnosed with Behçet's disease according to the Behçet's Syndrome International Study Group criteria and then managed with the prophylactic low molecular weight heparin, cyclophosphamide, and prednisolone, resulting in significant improvement in the patient's symptoms. Presentation with cerebral venous thrombosis, pulmonary thrombosis, and aneurysm simultaneously is very rare in Behçet's disease. This made this case distinct and challenging in achieving good control of thrombosis and aneurysm simultaneously, which needs close monitoring and a multidisciplinary team to deal with the case.