Alekberova Z S, Ovcharov P S, Lisitsyna T A, Volkov A V, Popkova T V
V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia.
Ter Arkh. 2017;89(5):79-82. doi: 10.17116/terarkh201789579-82.
Behçet's disease (BD) is systemic vasculitis of unknown etiology, which is more common in the countries located along the Great Silk Road. The disease is diagnosed if a patient has 4 key diagnostic signs: aphthous stomatitis, genital sores, and eye and skin lesions. Vascular diseases referred to as minor criteria for BD are characterized by the formation of aneurysms and thrombosis, predominantly in the venous bed. In venous disorders, a blood clot can form in any vessel, including caval, cerebral, pulmonary, and other veins. The paper describes two clinical cases of BD with intracardiac thrombosis. In one case, a 24-year-old male patient with a documented diagnosis of BD, echocardiography revealed a left ventricular spontaneous echo contrast phenomenon that disappeared due to immunosuppressive therapy. The other case was a 34-year-old female patient, in whom the diagnosis was based on the international disease criteria: aphthous stomatitis, skin lesions (pseudopustulosis, erythema nodosum), and genital sores. Computed tomographic angiography showed a 3.7×2.2-cm mass (thrombus) in the right atrium. In addition, blood clots were present in the hepatic and inferior vena cava. No abnormalities in the coagulation system were found in both cases.
白塞病(BD)是一种病因不明的系统性血管炎,在丝绸之路沿线国家更为常见。如果患者出现4个关键诊断体征:口腔溃疡、生殖器溃疡以及眼部和皮肤病变,则可诊断为该病。作为BD次要标准的血管疾病的特征是动脉瘤和血栓形成,主要发生在静脉床。在静脉疾病中,血凝块可在任何血管中形成,包括腔静脉、脑静脉、肺静脉和其他静脉。本文描述了两例白塞病合并心内血栓形成的临床病例。其中一例为一名24岁男性患者,确诊为白塞病,超声心动图显示左心室自发显影现象,经免疫抑制治疗后消失。另一例为一名34岁女性患者,其诊断基于国际疾病标准:口腔溃疡、皮肤病变(假脓疱病、结节性红斑)和生殖器溃疡。计算机断层血管造影显示右心房有一个3.7×2.2厘米的肿块(血栓)。此外,肝静脉和下腔静脉也有血凝块。两例患者均未发现凝血系统异常。