Adeeb Fahd, Stack Austin G, Fraser Alexander D
Department of Rheumatology, University Hospital Limerick, Limerick, Ireland.
Graduate Entry Medical School, University of Limerick, Limerick, Ireland.
Int J Rheumatol. 2017;2017:2160610. doi: 10.1155/2017/2160610. Epub 2017 Sep 10.
Behçet's disease (BD) is a chronic relapsing vasculitis that affects vessels of all types and sizes with a broad spectrum of phenotypic heterogeneity and complex immunopathogenesis. Efforts by the scientific community to resolve the unmet needs of BD and gaps in our knowledge have been hampered by considerable challenges that primarily relate to the rare nature of the disease in many parts of the world and its heterogeneity. Controversies remain in many aspects of the disease including the diagnostic criteria, immunopathogenesis and biomarker discovery, geographical variation, and therapeutic considerations. In this review, we highlight recent advances in our scientific understanding of BD, shed new insights into diagnostic and treatment strategies, and discuss residual gaps in our knowledge that will serve as the basis for current and future research.
白塞病(BD)是一种慢性复发性血管炎,可累及各种类型和大小的血管,具有广泛的表型异质性和复杂的免疫发病机制。科学界为满足白塞病未被满足的需求和填补知识空白所做的努力,受到了诸多挑战的阻碍,这些挑战主要与该疾病在世界许多地区的罕见性及其异质性有关。在该疾病的许多方面仍存在争议,包括诊断标准、免疫发病机制和生物标志物发现、地域差异以及治疗考量。在本综述中,我们重点介绍了对白塞病科学认识的最新进展,为诊断和治疗策略提供了新的见解,并讨论了我们知识中仍存在的空白,这些空白将作为当前和未来研究的基础。