Gewrichtsschade ten gevolge van hemofilie

A spontaneous bleeding tendency is the hallmark of severe haemophilia, with a predilection for the large synovial joints. Joint bleeds are painful, have a major
impact on quality of life, and cause damage to the synovium, cartilage, and subchondral bone. The aim of this review is to describe the mechanism behind blood-induced joint damage, current and potential treatment strategies. Blood-exposure induces synovial inflammation, leading to the formation of cartilage-degrading factors. Moreover, inflammation in combination with iron induces synovial proliferation and neovascularization. This makes the synovial tissue
vulnerable to a rebleed, and can induce a vicious cycle of bleeding – synovitis – bleeding. Cartilage damage results indirectly from synovial changes, but also
directly from blood-exposure. Iron and activated macrophages cause cartilage degeneration and chondrocyte apoptosis. Bone changes characteristic
for haemophilic arthropathy are osteoporosis, cyst formation, and osteophyte formation. Treatment of joint damage in haemophilia is multidisciplinary and consists of prevention via clotting factor substitution, symptomatic treatment, maintaining functionality, and orthopaedic surgery. Disease-modifying interventions for blood-induced joint damage are an area of intensive research, but these treatments are not investigated in clinical trials yet.