Kötter Ina, Lötscher Fabian
Division of Rheumatology and Inflammatory Rheumatic Diseases, University Hospital Hamburg Eppendorf and Clinic for Rheumatology and Immunology Bad Bramstedt, Bad Bramstedt, Germany.
Department of Rheumatology and Immunology, Inselspital Bern, University of Bern, Bern, Switzerland.
Front Med (Lausanne). 2021 Apr 9;8:639758. doi: 10.3389/fmed.2021.639758. eCollection 2021.
Behçet's Syndrome (BS) is a variable vessel vasculitis according to the Chapel Hill Consensus Nomenclature (1) and may thus affect any organ, including major and minor arterial and venous vessels to a varying degree and with varying frequency. Although the main features of BS are recurrent oral and genital aphthous ulcers, cutaneous lesions, ocular inflammation and arthritis-major vessel and life-or organ threatening involvement of internal organs and the central and peripheral nervous system occur. In general, BS in Europe appears to form six phenotypes of clinical manifestations (2), which are (1) mucocutaneous only, (2) predominant arthritis/articular involvement, (3) vascular phenotype, (4) ocular manifestations, which are most likely associated with CNS manifestations and HLA-B51, (5) dominant parenchymal CNS manifestations (being associated with the ocular ones), and (6) gastrointestinal involvement. Mucocutaneous manifestations are present in almost all patients/all phenotypes. In the following review, we summarize the current knowledge concerning vascular, neurologic, gastrointestinal and musculoskeletal manifestations of the disease.
根据《 Chapel Hill 共识命名法》(1)，白塞病(BS)是一种可变血管性血管炎，因此可能影响任何器官，包括大小动静脉血管，程度和频率各不相同。虽然白塞病的主要特征是复发性口腔和生殖器阿弗他溃疡、皮肤病变、眼部炎症和关节炎，但主要血管以及危及生命或器官的内脏和中枢及周围神经系统受累也会发生。一般来说，欧洲的白塞病似乎形成六种临床表现型(2)，分别是：(1)仅黏膜皮肤型，(2)以关节炎/关节受累为主型，(3)血管型，(4)眼部表现型，最可能与中枢神经系统表现和 HLA-B51 相关，(5)以实质性中枢神经系统表现为主型(与眼部表现相关)，以及(6)胃肠道受累型。几乎所有患者/所有表型都有黏膜皮肤表现。在以下综述中，我们总结了有关该疾病血管、神经、胃肠道和肌肉骨骼表现的当前知识。
