Arthritis and Tissue Degeneration Program, Hospital for Special Surgery, New York, New York.
Department of Pathology and Laboratory Medicine, Hospital for Special Surgery, New York, New York.
Semin Thromb Hemost. 2015 Nov;41(8):832-7. doi: 10.1055/s-0035-1564445. Epub 2015 Oct 9.
Hemophilia A is an X-linked bleeding disorder that can be largely controlled by treatment with recombinant factor VIII. However, this treatment is only partially effective in preventing hemophilic arthropathy (HA), a debilitating degenerative joint disease that is caused by intra-articular bleeding events. The disease progression of HA has several distinct steps, beginning with hemophilic synovitis (HS), a hyperplasia of the synovial lining coupled with a neovascular response, followed by joint erosion with cartilage destruction and erosion of the underlying bone. The early stages of HA have certain features in common with arthritides such as rheumatoid arthritis (RA), whereas the later degenerative stages of HA have some similarities with osteoarthritis (OA). The main purpose of this review is to explore the similarities between HA with RA and OA and discuss how this information could potentially help understand the pathogenesis of HA and uncover new treatment opportunities.
血友病 A 是一种 X 连锁出血性疾病,可通过重组因子 VIII 治疗得到有效控制。然而,这种治疗方法仅能部分预防血友病性关节炎(HA),HA 是一种进行性关节疾病,由关节内出血事件引起。HA 的疾病进展有几个不同的阶段,首先是血友病性滑膜炎(HS),滑膜衬里的增生伴随着新生血管反应,随后是关节侵蚀、软骨破坏和骨下骨侵蚀。HA 的早期阶段与类风湿关节炎(RA)等关节炎有某些共同特征,而 HA 的晚期退行性阶段与骨关节炎(OA)有一些相似之处。本综述的主要目的是探讨 HA 与 RA 和 OA 的相似之处,并讨论这些信息如何有助于深入了解 HA 的发病机制并为其治疗提供新的机会。