Hemophilic arthropathy in patients with von Willebrand disease.

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.