Alpsoy Erkan, Leccese Pietro, Emmi Giacomo, Ohno Shigeaki
Department of Dermatology and Venereology, School of Medicine, Akdeniz University, Antalya, Turkey.
Rheumatology Department of Lucania, Rheumatology Institute of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza and Matera, Italy.
Front Med (Lausanne). 2021 Apr 28;8:624795. doi: 10.3389/fmed.2021.624795. eCollection 2021.
Behçet's disease (BD) is a chronic, relapsing inflammatory, multisystem disease of unknown etiology. The disease has a wide clinical spectrum of mucocutaneous lesions and ocular, vascular, articular, neurologic, gastrointestinal and cardiac involvement. Although the number of effective drugs used in the disease's treatment has increased in recent years, BD is still associated with severe morbidity because of mainly mucocutaneous, articular and ocular symptoms and an increased mortality because of large vessel, neurological, gastrointestinal and cardiac involvement. Many factors are associated with a more serious course, such as male gender and a younger age of onset. While the severity of the disease is more pronounced in the first years of the disease, it decreases in most patients after the age of forties. The primary goal of treatment should be the prevention of irreversible organ damage. Therefore, early diagnosis and appropriate treatment and close follow-up are mandatory to reduce the morbidity and mortality of the disease. Treatment varies depending on the organ involved and the severity of the involvement. For all these reasons, the treatment should be personalized and arranged with a multidisciplinary approach according to the organs involved. Treatment is mainly based on suppression of the inflammatory attacks of the disease using local and systemic immunomodulatory and immunosuppressive drugs. In this review, based on the mainly controlled studies and personal experience in clinical practice and basic research in this field, we propose a stepwise, symptom-based, algorithmic approach for the management of BD with a holistic perspective.
白塞病（BD）是一种病因不明的慢性、复发性炎症性多系统疾病。该疾病具有广泛的临床谱，包括黏膜皮肤病变以及眼部、血管、关节、神经、胃肠道和心脏受累。尽管近年来用于该疾病治疗的有效药物数量有所增加，但由于主要的黏膜皮肤、关节和眼部症状，BD仍与严重的发病率相关，并且由于大血管、神经、胃肠道和心脏受累，死亡率也有所增加。许多因素与更严重的病程相关，如男性性别和发病年龄较轻。虽然疾病的严重程度在疾病的最初几年更为明显，但在大多数40岁以后的患者中会有所减轻。治疗的主要目标应该是预防不可逆的器官损伤。因此，早期诊断、适当治疗和密切随访对于降低该疾病的发病率和死亡率至关重要。治疗因受累器官和受累严重程度而异。基于所有这些原因，治疗应该个性化，并根据受累器官采用多学科方法进行安排。治疗主要基于使用局部和全身免疫调节及免疫抑制药物来抑制疾病的炎症发作。在本综述中，基于该领域主要的对照研究以及临床实践和基础研究中的个人经验，我们从整体角度提出一种逐步的、基于症状的、算法化的白塞病管理方法。
