Department of Haematology/Van Creveldkliniek, University Medical Center Utrecht, The Netherlands.
Blood Rev. 2012 Nov;26(6):261-6. doi: 10.1016/j.blre.2012.09.002. Epub 2012 Sep 23.
von Willebrand disease (VWD) affects approximately 1% of the population. Joint bleeds are not a predominant symptom of VWD, however they do occur. Especially in patients with severe VWD, joint bleeds have been described in up to 45% of the patients. Recurrent hemarthroses can lead to joint destruction which is the main cause of morbidity, as is observed in hemophilia patients. In VWD however, the occurrence, severity, onset, treatment and impact of arthropathy have not been well described. This literature review summarizes all publications on arthropathy in patients with hereditary VWD, published until 1-5-2012. Joint bleeds in VWD are associated with low FVIII levels and seem to occur at young age. Depending on the severity of VWD, arthropathy occurs in 2-30% of the patients. Radiological joint damage can be seen but has not systematically been studied. Arthropathy can affect daily functioning and interfere with the quality of life. For acute management of joint bleeds FVIII/VWF concentrates can be used in VWD patients unresponsive to desmopressin or desmopressin in responsive VWD patients. When severe joint bleeds have caused synovitis and arthropathy, chemical or radioactive synovectomy and surgical joint procedures seem feasible to prevent further joint damage. Additional research is needed to learn more about the severity, onset and impact of arthropathy in VWD. Ongoing studies will hopefully answer the question whether prophylactic treatment with coagulation factor concentrates should be considered standard of care in severe VWD to prevent arthropathy.
血管性血友病（VWD）影响大约 1%的人口。关节出血并不是 VWD 的主要症状，但确实会发生。尤其是在严重 VWD 的患者中，高达 45%的患者出现过关节出血。反复发生的关节积血可导致关节破坏，这是导致发病率的主要原因，就像血友病患者一样。然而，在 VWD 中，关节病的发生、严重程度、发病、治疗和影响尚未得到很好的描述。这篇文献综述总结了截至 2012 年 5 月 1 日发表的所有关于遗传性 VWD 患者关节病的出版物。VWD 中的关节出血与 FVIII 水平低有关，似乎发生在年轻时期。根据 VWD 的严重程度，2-30%的患者会发生关节病。可以看到放射学关节损伤，但尚未进行系统研究。关节病会影响日常功能，并干扰生活质量。对于急性关节出血的管理，FVIII/VWF 浓缩物可用于对去氨加压素无反应或对去氨加压素有反应的 VWD 患者。当严重的关节出血引起滑膜炎和关节病时，化学或放射性滑膜切除术以及手术关节程序似乎可以预防进一步的关节损伤。需要进一步的研究来了解 VWD 中关节病的严重程度、发病和影响。正在进行的研究有望回答是否应考虑在严重 VWD 中预防性使用凝血因子浓缩物作为预防关节病的标准治疗方法。
