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本病例摘自2013年11月21日刊《新英格兰医学杂志》： Endocardial Calcification in Beh?et's Disease 白塞病的心内膜钙化 A 68-year-old man who had Beh?et's disease with a 30-year history of oral and genital ulcers and erythema nodosum presented with progressive leg edema and dyspnea. The physical examination revealed varices of the chest and abdominal wall (Panel A), caused by collateral circulation associated with obstruction of the superior vena cava (SVC). Chest computed tomography (CT) showed SVC obstruction (Panel B, arrows) and dilatation of the inferior vena cava (arrowhead). Endocardial calcification on the midlateral and apical wall (Panel C, arrow) was more extensive than that seen on imaging performed 11 years earlier (Panel D, arrow), and there was increased dilatation of the right ventricle and both atria. A restrictive cardiomyopathy with endomyocardial fibrosis, which can be a complication of Beh?et's disease, was diagnosed on the basis of echocardiography (video) and cardiac catheterization (including endomyocardial biopsy). After diuresis, the edema and dyspnea improved, but the varices of the trunk and lower limbs remained. 68岁男，既往白塞病，口腔和生殖器溃疡合并结节性红斑30年，因腿部进行性水肿合并呼吸困难求诊。查体发现胸壁和腹壁可见明显静脉曲张（图A），与上腔静脉（SVC）闭塞相关的侧枝循环有关。胸部CT检查证实SVC闭塞（图B，箭头）和下腔静脉扩张（箭头）。与11年前的影像学资料相比（图D），心内膜中侧壁和顶壁的钙化（图C，箭头）累及范围更广。右心室和双侧心房无继续扩张。根据超声心动图（请看视频）和心导管检查（包括心内膜活检）等结果，我们诊断为限制性心肌病合并心内膜心肌纤维化，系白塞病的并发症。利尿治疗后，水肿和呼吸困难出现缓解，但躯干和下肢的静脉曲张未见改善。 视频： |
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