Evereklioglu Cem
Department of Ophthalmology, Erciyes University Medical Faculty, Kayseri, Turkey.
Surv Ophthalmol. 2005 Jul-Aug;50(4):297-350. doi: 10.1016/j.survophthal.2005.04.009.
Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce a wide variety of symptoms. In mild cases, orogenital ulcers and skin lesions are the only findings during the entire clinical course, whereas ocular lesions that occur in more than half of the cases, frequently bilateral, can eventually lead to blindness. Pulmonary, gastrointestinal, and central nervous system involvements may sometimes be life-threatening. This review examines the epidemiology, frequency, immunology, and immunohistopathology of Behçet disease with recent theories of several agents, including phosphoantigens, superantigens, heat-shock proteins, and adenosine deaminase. Perspectives on the possible roles of new etiopathological molecules, such as nitric oxide, endothelin, and homocysteine, are presented. Ocular and systemic clinical features, diagnostic criteria, classifications, laboratory, fundus fluorescein angiography, and radiologic imaging are discussed. Differential diagnosis, disease in pregnancy and childhood, and prognosis with regard to Behçet-induced systemic and ocular complications are also evaluated. Traditional and current treatments with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil are summarized and recent insights into the pharmacology and effects of thalidomide, tacrolimus (FK-506), interferon-alpha, anti-TNF-alpha blocking monoclonal autoantibody (infliximab) and soluble TNF receptor (etanercept) are reviewed. Key clinical investigations with the status of ongoing clinical trials aimed at addressing the drug's efficacy, surgical care, and studies that have raised the possibility of new therapeutic uses are also presented. The challenges posed by the drug's teratogenicity and adverse effects are also considered, if present.
白塞病是一种特发性、多系统、慢性复发性疾病，其特征为病情加重与缓解期交替出现、发作性全葡萄膜炎，以及任何大小动脉和静脉的侵袭性非肉芽肿性闭塞性血管炎，并伴有主要累及眼部视网膜和前段血管系统的暴发性眼部炎症发作。该疾病以内皮功能障碍为特征，可产生多种症状。在轻症病例中，整个临床过程中仅出现口生殖器溃疡和皮肤病变，而超过半数病例出现的眼部病变（常为双侧）最终可导致失明。肺部、胃肠道和中枢神经系统受累有时可能危及生命。本综述探讨了白塞病的流行病学、发病率、免疫学和免疫组织病理学，并介绍了包括磷酸抗原、超抗原、热休克蛋白和腺苷脱氨酶在内的几种致病因素的最新理论。还阐述了一氧化氮、内皮素和同型半胱氨酸等新的病因病理分子可能发挥的作用。讨论了眼部和全身临床特征、诊断标准、分类、实验室检查、眼底荧光血管造影和影像学检查。还评估了鉴别诊断、妊娠和儿童期疾病，以及白塞病所致全身和眼部并发症的预后。总结了局部、眼周和全身使用皮质类固醇、秋水仙碱、氨苯砜、环孢素、硫唑嘌呤、甲氨蝶呤、环磷酰胺和苯丁酸氮芥的传统及当前治疗方法，并综述了沙利度胺、他克莫司（FK-506）、干扰素-α、抗TNF-α阻断单克隆自身抗体（英夫利昔单抗）和可溶性TNF受体（依那西普）的药理学及作用的最新见解。还介绍了旨在探讨药物疗效的关键临床研究、临床试验现状、手术治疗，以及提出新治疗用途可能性的研究。如有药物致畸性和不良反应带来的挑战，也予以考虑。
