K.P.M. van Galen MD, MSc, E.P. Mauser-Bunschoten MD, PhD
SUMMARY
Von Willebrand disease is the most common congenital bleeding disorder characterised by mucocutaneous bleeding. However, joint bleeds also occur in a significant proportion of patients with severe Von Willebrand disease. Until recently, joint bleeding did not get much attention in clinical research on Von Willebrand disease, despite the fact that recurrent joint bleeds may lead to arthropathy. Arthropathy in Von Willebrand disease has a negative impact on joint function, participation and quality-of-life. Risk factors are a low FVIII level and a history of more than five joint bleeds. Arthropathy in Von Willebrand disease can be measured using the Haemophilia Joint Health Score and the joint X-ray Pettersson score. The value of magnetic resonance imaging or ultrasound to detect early arthropathy in Von Willebrand disease remains to be determined. The Haemophilia Activities List questionnaire is feasible to quantify functional abilities in Von Willebrand disease. The most important measure to prevent arthropathy is to prevent joint bleeding. Clotting factor prophylaxis has proven very effective to do so. Since there is no general consensus to guide the use of prophylaxis in Von Willebrand disease, the decision on when and how to start prophylaxis is based on individual patient’s assessments by a haemophilia treatment centre. Future studies in Von Willebrand disease arthropathy could address the optimal timing and schedule of prophylaxis, optimal treatment of an acute joint bleed to prevent arthropathy, effectiveness of rehabilitation programs, orthopaedic surgery and the clinical use of measurement instruments. Prospective joint assessments and registration of joint bleeds in future population studies on severe Von Willebrand disease will be a good starting point.
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