Alpsoy Erkan
Department of Dermatology and Venerology, Akdeniz University School of Medicine, 07059 Antalya, Turkey.
Patholog Res Int. 2012;2012:871019. doi: 10.1155/2012/871019. Epub 2011 Oct 5.
Behçet's disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. The disease is much more frequent along the ancient "Silk Route" extending from Eastern Asia to the Mediterranean basin, compared with Western countries. The disease usually starts around the third or fourth decade of life. Male sex and a younger age of onset are associated with more severe disease. Although the treatment has become much more effective in recent years, BD is still associated with severe morbidity and considerable mortality. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early, and appropriate treatment is mandatory to reduce morbidity and mortality. The treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. In this paper, current state of knowledge regarding the therapeutic approaches is outlined. To provide a rational framework for selecting the appropriate therapy along the various treatment choices, a stepwise, symptom-based, evidence-based algorithmic approach was developed.
白塞病（BD）是一种病因不明的慢性、复发性、使人衰弱的全身性血管炎，具有黏膜皮肤病变、眼部、血管、关节、神经、胃肠道、泌尿生殖系统和肺部受累的临床特征。与西方国家相比，该病在从东亚延伸至地中海盆地的古代“丝绸之路”沿线更为常见。该病通常在人生的第三个或第四个十年左右开始发病。男性和较年轻的发病年龄与更严重的病情相关。尽管近年来治疗已变得更加有效，但白塞病仍与严重的发病率和相当高的死亡率相关。治疗的主要目标应是预防不可逆的器官损害。因此，密切监测、早期和适当治疗对于降低发病率和死亡率是必不可少的。治疗主要基于使用免疫调节和免疫抑制药物抑制疾病的炎症发作。本文概述了有关治疗方法的当前知识状态。为了在各种治疗选择中提供一个选择合适治疗方法的合理框架，开发了一种基于症状、循证的逐步算法方法。
