Concurrent pulmonary artery aneurysms and intracardiac thrombus in a Caucasian man with Behçet’s disease: case report and literature review

Behçet’s disease (BD) is a systemic inflammatory disease marked by oral and genital ulcers. While pulmonary arterial aneurysms (PAAs) are not a common clinical manifestation of BD, they are a leading cause of death. We describe a case of recurrent pulmonary aneurysms and intracardiac thrombus in a young Caucasian man who met Japanese diagnostic criteria for BD. He had multiple recurrences of haemoptysis from PAAs despite treatment with high-dose glucocorticoids and cyclophosphamide. He ultimately achieved remission on infliximab. A review of the English literature from 1980–2016 was conducted for cases of concurrent pulmonary artery aneurysm and extrapulmonary thrombosis complicating BD. 38 relevant cases were found and details of the cases were extracted and compiled. PAAs associated with BD are most commonly seen in young men of Middle Eastern descent, and mortality remains high despite treatment, with 62% five-year survival rates. PAAs may be associated with concurrent venous thrombotic events, including intracardiac thrombosis. In such cases, immunosuppressive therapy should be initiated and anticoagulation avoided or administered with extreme caution due to the high risk of bleeding. Although the findings of PAA and intracardiac thrombus, as well as BD itself, are rare in Caucasian individuals, a high index of suspicion is still warranted for cases of haemoptysis with associated imaging findings consistent with aneurysms of the pulmonary vasculature.