Ambrosino Valeria, De Marco Francesca, Valli Gabriele, Ruggieri Maria Pia, Morelli Sergio
Postgraduate School of Internal Medicine, Sapienza University of Rome, 00185 Rome, Italy.
Emergency Department, San Giovanni Addolorata Hospital, 00184 Rome, Italy.
Eur Heart J Case Rep. 2024 Sep 3;8(9):ytae467. doi: 10.1093/ehjcr/ytae467. eCollection 2024 Sep.
Behçet's disease is an inflammatory condition, caused by vasculitis of big and small veins and arteries in which, although vascular inflammation is the basis of disease, cardiac involvement is rare. We present a rare case of a man, affected by Behçet's disease, with pulmonary embolism due to a floating thrombus in the right ventricle.
We report a case of a 36-year-old man admitted to emergency department due to dyspnoea and haemoptysis. He had already been diagnosed with Behçet's disease, and he was in therapy with low doses of azathioprine and prednisone from three months. Thorax CT scan detected pulmonary embolism with pulmonary infraction. No evidence of deep vein thrombosis was found. The echocardiogram pointed out a floating mass of at least 30 mm in the right ventricle. Cardiac magnetic resonance confirmed the diagnosis of right ventricle thrombosis. On the hypothesis of an inflammatory genesis of the thrombosis, immunosuppressive drugs and anticoagulation with vitamin K antagonist were prescribed. The patient underwent echocardiograms every 3 weeks, and the mass disappeared 5 months later.
Behçet's disease is a systemic inflammatory disorder that often affects vessels and rarely the heart. Thrombosis can be the only clinical feature of primary or relapsing events with also atypical origin site. Thrombosis suggests a high inflammatory status that needs to be balanced with the right immunosuppressive therapy, associated to anticoagulation.
白塞病是一种炎症性疾病,由大小动静脉的血管炎引起,尽管血管炎症是该疾病的基础,但心脏受累情况罕见。我们报告一例患有白塞病的男性罕见病例,其因右心室漂浮血栓导致肺栓塞。
我们报告一例36岁男性因呼吸困难和咯血入住急诊科。他已被诊断为白塞病,且已接受低剂量硫唑嘌呤和泼尼松治疗三个月。胸部CT扫描检测到肺栓塞伴肺梗死。未发现深静脉血栓形成的证据。超声心动图显示右心室内有一个至少30毫米的漂浮团块。心脏磁共振成像证实了右心室血栓形成的诊断。基于血栓形成的炎症起源假说,给予免疫抑制药物和维生素K拮抗剂抗凝治疗。患者每3周接受一次超声心动图检查,5个月后团块消失。
白塞病是一种全身性炎症性疾病,常累及血管,很少累及心脏。血栓形成可能是原发性或复发性事件的唯一临床特征,其起源部位也可能不典型。血栓形成提示炎症状态较高,需要通过适当的免疫抑制治疗并联合抗凝治疗来平衡。