title: Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study.
abstract: 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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: K P M
authors: KPM
authors: van Galen
authors: Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors: Y V
authors: YV
authors: Sanders
authors: Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors: U
authors: U
authors: Vojinovic
authors: Department of Thrombosis and Haemostasis and Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, Leiden, The Netherlands.
authors: J
authors: J
authors: Eikenboom
authors: Department of Pediatric Haematology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors: M H
authors: MH
authors: Cnossen
authors: Department of Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: R E G
authors: REG
authors: Schutgens
authors: Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, The Netherlands.|Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.
authors: J G
authors: JG
authors: van der Bom
authors: Department of Pediatric Haematology, Academisch Medisch Centrum, Emma Children's Hospital, Amsterdam, The Netherlands.
authors: K
authors: K
authors: Fijnvandraat
authors: Department of Haematology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors: B A P
authors: BAP
authors: Laros-Van Gorkom
authors: Department of Haematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors: K
authors: K
authors: Meijer
authors: Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors: F W G
authors: FWG
authors: Leebeek
authors: Department of Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors: E P
authors: EP
authors: Mauser-Bunschoten
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D000369:Aged, 80 and over; D001780:Blood Coagulation Tests; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D003430:Cross-Sectional Studies; D005260:Female; D006395:Hemarthrosis / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology* / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D017063:Outcome Assessment, Health Care; D015995:Prevalence; D011788:Quality of Life; D011795:Surveys and Questionnaires; D055815:Young Adult; D014842:von Willebrand Diseases / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor
keywords: arthropathy; joint bleeds; joint damage; joint pain; quality of life; von Willebrand disease