Störk Stefan, Kneitz Christian, Bröcker Eva-B, Hoyer Caroline, Ertl Georg, Angermann Christiane E
Medizinische Klinik und Poliklinik I, Universität Würzburg, Klinikstrasse 6-8, Würzburg.
Med Klin (Munich). 2008 Mar 15;103(3):143-52. doi: 10.1007/s00063-008-1021-3.
Behçet's disease is a chronic relapsing systemic vasculitis of unknown etiology, affecting predominantly oral and genital mucocutaneous tissues and also the eyes. The disease is spread worldwide with a higher prevalence rate in countries along the ancient Silk Route, but it is rare (1-10/100,000) in Central and Northern Europe. Genetic, environmental, immunologic, inflammatory and rheologic factors are involved in the pathogenesis and the course of the disease. Any vascularized organ may be affected. Eye involvement is frequent, and may eventually result in loss of vision. Further important complications are cerebral manifestations, thrombotic syndromes, and arterial aneurysms with a high risk of rupture. Diagnosis and therapy of Behçet's disease are best managed by an interdisciplinary team. Skin lesions may be controlled by systemic treatment with colchicine, alternatively with dapsone, and in severe cases with thalidomide. Active systemic disease should be treated more aggressively using immunosuppressants. Despite advances in treatment relapses are still frequent, and systemic disease remains associated with an adverse prognosis.
白塞病是一种病因不明的慢性复发性系统性血管炎，主要累及口腔和生殖器的黏膜皮肤组织以及眼睛。该病在全球范围内均有分布，在古代丝绸之路沿线国家的患病率较高，但在中欧和北欧较为罕见（1-10/10万）。遗传、环境、免疫、炎症和血液流变学因素参与了该病的发病机制和病程。任何血管化器官都可能受累。眼部受累很常见，最终可能导致视力丧失。其他重要并发症包括脑部表现、血栓形成综合征以及有高破裂风险的动脉瘤。白塞病的诊断和治疗最好由多学科团队进行管理。皮肤病变可通过秋水仙碱进行全身治疗控制，也可选用氨苯砜，严重病例可使用沙利度胺。活动性全身性疾病应使用免疫抑制剂进行更积极的治疗。尽管治疗取得了进展，但复发仍然频繁，全身性疾病仍然与不良预后相关。
