Kurokawa Manae S, Yoshikawa Hideshi, Suzuki Noboru
Department of Immunology, St. Marianna University School of Medicine, Kanagawa, Japan.
Semin Respir Crit Care Med. 2004 Oct;25(5):557-68. doi: 10.1055/s-2004-836147.
Behçet's disease (BD) is a systemic disorder characterized by recurrent attacks of acute inflammation. Major symptoms are oral aphthous ulcers, uveitis, skin lesions, and genital ulcerations. Involvement of vessels, gastrointestinal (GI) tract, and central nervous system (CNS) is less frequent but is associated with a poor prognosis. Pulmonary complications of BD include aneurysms of the aorta, great vessels, or pulmonary arteries; arterial or venous thrombosis; pulmonary parenchymal changes; pleurisy, and intracardiac thrombosis. Hemoptysis caused by pulmonary artery aneurysms may lead to lethal hemorrhage. Recent advances in therapeutic strategies have improved the prognosis. In this review, the salient clinical and histopathological features of BD and treatment strategies are discussed.
白塞病（BD）是一种以急性炎症反复发作为特征的全身性疾病。主要症状为口腔阿弗他溃疡、葡萄膜炎、皮肤病变和生殖器溃疡。血管、胃肠道（GI）和中枢神经系统（CNS）受累较少见，但预后较差。白塞病的肺部并发症包括主动脉、大血管或肺动脉的动脉瘤；动脉或静脉血栓形成；肺实质改变；胸膜炎和心内血栓形成。肺动脉瘤引起的咯血可能导致致命性出血。治疗策略的最新进展改善了预后。在这篇综述中，讨论了白塞病的显著临床和组织病理学特征以及治疗策略。
