Roosendaal Goris, Lafeber Floris P J G
Van Creveldkliniek and National Hemophilia Center, Division of Internal Medicine, University Medical Center Utrecht, The Netherlands.
Semin Thromb Hemost. 2003 Feb;29(1):37-42. doi: 10.1055/s-2003-37938.
Hemophilia is a X chromosome-linked disease characterized by an increased tendency to hemorrhage. Because of recurrent hemarthrosis, specific changes occur in synovium and cartilage. This process is called hemophilic arthropathy. The pathogenetic mechanisms involved are not precisely known. Current concepts, which are based on experimental in vitro studies and clinical experience, hold that the synovium becomes catabolically active because of the exposure to blood components and as a result induces cartilage destruction. A considerable number of reports concerning blood-induced joint damage suggest that synovial changes have a leading role in the development of the joint damage and therefore precede the changes in cartilage. However, there are also observations that question whether this is the only and initiating mechanism of joint damage in hemophilia. These observers hold that intra-articular blood has a direct harmful effect on cartilage before synovial changes and suggest that joint damage may occur before synovial inflammation is evident. Primarily, there may be damage of articular cartilage with synovitis as a consequence. Some studies show that synovitis is involved but that it is not the only mechanism in the joint damage caused by intra-articular bleeding. These findings do not contradict the current concept of blood-induced cartilage damage in which synovial changes are thought to play an important role. Several pathological processes are possibly involved, some of them occurring in parallel and others sequentially. Possibly, intra-articular blood first has a direct effect on cartilage, and then it affects the synovium. Thus, both processes occur in parallel, and although they influence each other they probably do not depend on each other. This concept resembles degenerative joint damage as found in osteoarthritis.
血友病是一种X染色体连锁疾病，其特征是出血倾向增加。由于反复发生关节积血，滑膜和软骨会出现特定变化。这个过程称为血友病性关节病。其中涉及的发病机制尚不完全清楚。基于体外实验研究和临床经验的当前观点认为，滑膜因接触血液成分而分解代谢活跃，从而导致软骨破坏。大量关于血液引起的关节损伤的报告表明，滑膜变化在关节损伤的发展中起主导作用，因此先于软骨变化。然而，也有观察结果质疑这是否是血友病关节损伤的唯一启动机制。这些观察结果认为，关节内血液在滑膜变化之前对软骨有直接有害作用，并表明关节损伤可能在滑膜炎症明显之前就已发生。主要是，可能先有关节软骨损伤，继而出现滑膜炎。一些研究表明滑膜炎参与其中，但它不是关节内出血导致关节损伤的唯一机制。这些发现与当前认为滑膜变化在血液引起的软骨损伤中起重要作用的观点并不矛盾。可能涉及几个病理过程，其中一些并行发生，另一些则相继发生。可能关节内血液首先对软骨有直接影响，然后影响滑膜。因此这两个过程并行发生，尽管它们相互影响，但可能并不相互依赖。这个概念类似于骨关节炎中发现的退行性关节损伤。
