ORIGINAL ARTICLE Von Willebrand disease Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study K. P. M. VAN GALEN,* Y. V. SANDERS, † U. VOJINOVIC, † J. EIKENBOOM, ‡ M. H. CNOSSEN,§ R. E. G. SCHUTGENS,* J. G. VAN DER BOM, ¶ k K. FIJNVANDRAAT,** B. A. P. LAROS-VAN GORKOM, †† K. MEIJER, ‡‡ F. W. G. LEEBEEK † and E. P. MAUSER-BUNSCHOTEN* FOR THE WIN STUDY GROUP *Department of Van Creveldkliniek, University Medical Center Utrecht, Utrecht; †Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam; ‡Department of Thrombosis and Haemostasis and Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, Leiden; §Department of Pediatric Haematology, Erasmus University Medical Center-Sophia Children’s Hospital, Rotterdam; ¶Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research; kDepartment of Clinical Epidemiology, Leiden University Medical Center, Leiden; **Department of Pediatric Haematology, Academisch Medisch Centrum Emma Children’s Hospital, Amsterdam; ††Department of Haematology, Radboud University Medical Center, Nijmegen; and ‡‡Department of Haematology, University Medical Center Groningen University of Groningen, Groningen, The Netherlands Background: 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. Objectives: 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. Methods: In the Willebrand in the Netherlands study 804 moderate and severe VWD patients [von Willebrand factor (VWF) activity ≤30U dL 1 ] 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. Results: Of all VWD patients 23% (184/804) self-reported JB. These 184 patients reported joint damage more often (54% vs. 18%, P < 0.001) and had lower HR-QoL (SF36, P < 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 1 year 1 , P < 0.001), more often had X-ray joint damage (44% vs. 11%, P = 0.001] and chronic joint pain (44% vs. 18%, P = 0.008) compared to 55 control VWD patients without JB. Conclusion: 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. Keywords: arthropathy, joint bleeds, joint damage, joint pain, quality of life, von Willebrand disease Correspondence: Karin P. M. van Galen, MD, Department of Haematology/Van Creveldkliniek, University Medical Center Utrecht, Heidelberglaan 100, Room C01.425, P.O. Box 85500, 3508 GA Utrecht, The Netherlands. Tel.: +31 8 875 584 50; fax: +31 88 75 554 38; e-mail: k.p.m.vangalen@umcutrecht.nl The findings in this manuscript have partly been presented at the 6th Annual Congress of the European Association for Haemophilia and Allied Disorders 2013 on February 6–8 in Warsaw, Poland (Winner First Abstract Prize); at the 24th congress of the International Society on Thrombosis and Haemostasis 2013 on June 26–July 4 in Amsterdam, The Netherlands (e-poster A-session); at the 19th con- gress of the European Hematology Association 2014 on June 12–15 in Milan, Italy (oral presentation). Accepted after revision 20 February 2015 © 2015 John Wiley & Sons Ltd e185 Haemophilia (2015), 21, e185–e192 DOI: 10.1111/hae.12670