There are no data on joint health and the influence of arthropathy on daily life activities in VWD. 4 However, hardly any information is available on the existence and severity of blood-induced arthropathy in VWD. 3 Joint bleeding occurs in half of the patients with type 3 VWD and in 5–10% of moderate and severe type 1 and type 2 VWD patients. In hemophilia patients with FVIII or FIX deficiency, recurrent joint bleeds are the main cause of pain and functional limitations, which is known as hemophilic arthropathy. 2 Low FVIII occurs because its chaperone protein VWF, which protects FVIII from degradation, is (partly) missing. Joint bleeds also occur in VWD, predominantly in severely affected patients with concomitant low Factor VIII levels (FVIII). 1 VWD is caused by a deficiency (type 1), dysfunction (type 2) or absence (type 3) of von Willebrand factor (VWF) and is mainly associated with mucocutaneous bleeding and menorrhagia. Von Willebrand disease (VWD) is a congenital bleeding disorder with a population prevalence of 0.6–1.3%. In conclusion, arthropathy occurs in 40% of VWD patients after joint bleeds and is associated with pain, radiological abnormalities, functional limitations, and less social participation ( Dutch trial register: NTR4548). 3 of 28, P<0.01, and median score on the participation questionnaire 6.1 vs. Arthropathy occurred in 40% of the cases versus 10% of the controls ( P3: 13 of 19 vs. We included 48 patients with verified joint bleeds (cases) and 48 controls: 60% males, mean age 46 years (range 18–80), median von Willebrand Factor activity 5 versus 8 IU/dL and Factor VIII 24 versus 36 IU/dL. Arthropathy was defined as a Hemophilia Joint Health Score of 10 or higher, or a Pettersson score over 3 of at least one joint. Assessments included the Hemophilia Joint Health Score (0–124), Pettersson score (0–13 per joint X-ray), Hemophilia Activity List score (0–100), joint pain (Visual Analog Scale 0–10), and the Impact on Participation and Autonomy questionnaire (0–20). VWD patients with and without verified joint bleeds were matched for age, sex and Factor VIII level or von Willebrand Factor activity in a nested case-control study within the Willebrand in the Netherlands study. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. Information on its prevalence and severity is limited. Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds.
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