Rosenstingl S, Dupuy E, Alves O, George B, Tobelem G
Service d'angiohématologie clinique, hôpital Lariboisière, 2, rue Ambroise-Paré, 75010 Paris, France.
Rev Med Interne. 2001 Feb;22(2):177-82. doi: 10.1016/s0248-8663(00)00308-8.
Mostly venous (95% of all vascular complications), and less frequently arterial (2 to 7% of all cases), vascular complications are commonplace in Behçet's disease (23 to 64% of the patients, depending on the series). Arterial complications are stenosis, occlusions and especially severe due to their unpredictable rupture risk, aneurysms. Intracranial aneurysms associated with Behçet's disease are exceptional. Until now, only ten cases have been published.
We report the case of a 36-year-old patient of Armenian origin in whom the diagnosis of Behçet's disease was made after a subarachnoid hemorrhage caused by the rupture of a left superior cerebellar artery aneurysm. The endovascular treatment of the aneurysm was associated with an immunosuppressive treatment consisting of cyclophosphamide, corticoids and colchicine. Within a 6-month period of follow up the evolution has been favorable. This is the first published case report of Behçet's disease associated with an aneurysm of the posterior circulation treated endovascularly. A review of the literature is also included.
Intracranial aneurysms are an exceptional but nevertheless severe localization of vascular complications in Behçet's disease. As in all other arterial lesions, recurrences are frequent. The treatment involves surgical or endovascular treatment that should be associated with corticoids and immunosuppressive therapy. Colchicine is useful for the prevention of relapses.
血管并发症在白塞病中很常见（占所有患者的23%至64%，具体比例取决于不同系列研究），其中大多数为静脉性并发症（占所有血管并发症的95%），动脉性并发症较少见（占所有病例的2%至7%）。动脉并发症包括狭窄、闭塞，尤其是动脉瘤，因其破裂风险不可预测，故而尤为严重。与白塞病相关的颅内动脉瘤极为罕见。截至目前，仅发表了10例相关病例。
我们报告一例36岁的亚美尼亚裔患者，该患者因左侧小脑上动脉动脉瘤破裂导致蛛网膜下腔出血后，被诊断为白塞病。动脉瘤的血管内治疗联合了由环磷酰胺、皮质类固醇和秋水仙碱组成的免疫抑制治疗。在6个月的随访期内，病情进展良好。这是首例关于经血管内治疗的后循环动脉瘤合并白塞病的病例报告。本文还包含了文献综述。
颅内动脉瘤是白塞病中血管并发症的一种罕见但严重的表现形式。与所有其他动脉病变一样，复发很常见。治疗方法包括手术或血管内治疗，且应联合皮质类固醇和免疫抑制治疗。秋水仙碱有助于预防复发。
