Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross‐sectional study. (9th April 2015)

• Record Type: Journal Article
• Authors: van Galen, K. P. M.; Sanders, Y. V.; Vojinovic, U.; Eikenboom, J.; Cnossen, M. H.; Schutgens, R. E. G.; van der Bom, J. G.; Fijnvandraat, K.; Laros‐Van Gorkom, B. A. P.; Meijer, K.; Leebeek, F. W. G.; Mauser‐Bunschoten, E. P.; the WiN Study Group
• Is Part Of: Haemophilia. Volume 21:Number 3(2015:May)
• Page Start: e185
• Page End: e192
• Publication Date: 2015-04-09
• Subjects: Hemophilia -- Periodicals; 616.1572005
• DOI: 10.1111/hae.12670
• Languages: English
• ISSNs: 1351-8216

Abstract

<abstract abstract-type="main" id="hae12670-abs-0011"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="hae12670-sec-0001" sec-type="section"> <title> <bold>Background:</bold> </title> <p>Joint bleeds (JB) are reported in a minority of patients with von Willebrand disease (VWD) but may lead to structural joint damage. Prevalence, severity and impact of JB in VWD are largely unknown.</p> </sec> <sec id="hae12670-sec-0002" sec-type="section"> <title> <bold>Objectives:</bold> </title> <p>The aim of this study was to assess JB prevalence, onset, treatment and impact on health‐related quality of life (HR‐QoL) and joint integrity in moderate and severe VWD.</p> </sec> <sec id="hae12670-sec-0003" sec-type="section"> <title> <bold>Methods:</bold> </title> <p>In the Willebrand in the Netherlands study 804 moderate and severe VWD patients [von Willebrand factor (VWF) activity ≤30U dL<sup>−1</sup>] completed a questionnaire on occurrence, sites and consequences of JB. To analyse JB number, onset, treatment and impact on joint integrity we additionally performed a patient–control study on medical file data comparing patients with JB to age, gender, factor VIII (FVIII)‐ and VWF activity matched VWD patients without JB.</p> </sec> <sec id="hae12670-sec-0004" sec-type="section"> <title> <bold>Results:</bold> </title> <p>Of all VWD patients 23% (184/804) self‐reported JB. These 184 patients reported joint damage more often (54% vs. 18%, <italic>P</italic> < 0.001) and had lower HR‐QoL (SF36, <italic>P</italic> < 0.05) compared to VWD patients not reporting JB. Of 55 patients with available JB data, 65% had the first JB before age 16. These 55 patients used more clotting factor concentrate (CFC; median dose 43 vs. 0 IE FVIII kg<sup>−1</sup> year<sup>−1</sup>, <italic>P</italic> < 0.001), more often had X‐ray joint damage (44% vs. 11%, <italic>P</italic> = 0.001] and chronic joint pain (44% vs. 18%, <italic>P</italic> = 0.008) compared to 55 control VWD patients without JB.</p> </sec> <sec id="hae12670-sec-0005" sec-type="section"> <title> <bold>Conclusion:</bold> </title> <p>In conclusion, joint bleeds are reported by 23% of moderate and severe VWD patients, mostly start in childhood, are associated with more CFC use, joint pain, lower HR‐QoL and significantly more radiological and self‐reported joint damage.</p> </sec> </abstract>