Matsumoto T, Uekusa T, Fukuda Y
First Department of Pathology, Juntendo University, School of Medicine, Tokyo, Japan.
Hum Pathol. 1991 Jan;22(1):45-51. doi: 10.1016/0046-8177(91)90060-3.
Behçet's disease associated with large vascular lesions is called vasculo-Behçet's disease. The pathologic features of eight autopsy cases of vasculo-Behçet's disease were studied. The patients' ages ranged from 31 to 56 years; there were five men and three women. Of these patients, large vascular lesions were as follows: aneurysm (three cases), aneurysm and arterial occlusion (one case), aneurysm with arterial and venous occlusion (one case), and venous occlusion (two cases). Aneurysms were of the saccular or dissecting type. The aneurysm formation most commonly occurred in the aorta (two cases). Histologically, aortitis was seen in six patients. Aortitis was divided into active and scar stages. In one case with active stage aortitis, granulomatous aortitis similar to Takayasu's arteritis was seen. In conclusion, we believe that the large arterial lesion in vasculo-Behçet's disease represents inflammation occurring in the media and adventitia. In the affected arteries, active arteritis occurs initially, followed by destruction of the media and fibrosis. Saccular aneurysms are probably produced by severe destruction of the media by intense active inflammation. The large venous lesions represent thrombophlebitis.
与大血管病变相关的白塞病称为血管型白塞病。对8例血管型白塞病尸检病例的病理特征进行了研究。患者年龄在31至56岁之间；男性5例，女性3例。这些患者的大血管病变如下：动脉瘤（3例）、动脉瘤合并动脉闭塞（1例）、动脉瘤合并动静脉闭塞（1例）和静脉闭塞（2例）。动脉瘤为囊状或夹层型。动脉瘤形成最常见于主动脉（2例）。组织学上，6例患者可见主动脉炎。主动脉炎分为活动期和瘢痕期。在1例活动期主动脉炎患者中，可见类似于大动脉炎的肉芽肿性主动脉炎。总之，我们认为血管型白塞病中的大动脉病变代表中膜和外膜发生的炎症。在受累动脉中，最初发生活动性动脉炎，随后中膜破坏和纤维化。囊状动脉瘤可能是由强烈的活动性炎症对中膜的严重破坏所致。大静脉病变代表血栓性静脉炎。
