Pleyer U, Hazirolan D, Winterhalter S, Stübiger N
Universitäts-Augenklinik, Uveitis Zentrum, Charité, Universitätsmedizin Berlin, Humboldt University, Augustenburger Platz 1, 13353, Berlin, Deutschland.
Ophthalmologe. 2013 Mar;110(3):273-84. doi: 10.1007/s00347-012-2780-z.
Behcet's disease (also called Admantiades-Behcet syndrome) is a chronic vasculitis. The disease is characterized by exacerbations and remissions of symptoms and organ manifestations and may produce only mild mucocutaneous lesions, whereas ocular lesions can cause blindness. In addition, involvement of the gastrointestinal tract, central nervous system (CNS) and large blood vessels is sometimes life-threatening. Cyclosporin A is the only agent for treatment of ocular lesions registered in Germany; however, the neurotoxicity and nephrotoxicity restrict usage of the drug. In patients suffering from severe uveitis, biologics have been a breakthrough. Interferon (IFN) alpha therapy has shown significant efficacy for intraocular inflammation. Monoclonal antibodies to TNF-alpha and interleukin-1 have been successful in clinical trials and are approved in some countries. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the therapy of Behcet's disease.
白塞病(也称为阿德曼蒂亚德斯 - 白塞综合征)是一种慢性血管炎。该疾病的特征是症状和器官表现的加重与缓解,可能仅产生轻度黏膜皮肤病变,而眼部病变可导致失明。此外,胃肠道、中枢神经系统(CNS)和大血管受累有时会危及生命。环孢素A是德国唯一注册用于治疗眼部病变的药物;然而,其神经毒性和肾毒性限制了该药物的使用。在患有严重葡萄膜炎的患者中,生物制剂带来了突破。α干扰素治疗已显示出对眼内炎症有显著疗效。抗TNF-α和白细胞介素-1的单克隆抗体在临床试验中取得成功,并在一些国家获得批准。本文总结了当前的知识状况,并强调了眼科医生在白塞病治疗中的重要作用。