Joint bleeds in von Willebrand disease patients have significant impact on quality of life and joint integrity: a cross-sectional study

Haemophilia. 2015 May;21(3):e185-e192. doi: 10.1111/hae.12670. Epub 2015 Apr 9.

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.

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.

Publication types

  • Multicenter Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Blood Coagulation Tests
  • Child
  • Child, Preschool
  • Comorbidity
  • Cross-Sectional Studies
  • Female
  • Hemarthrosis / diagnosis
  • Hemarthrosis / epidemiology*
  • Hemarthrosis / etiology*
  • Hemarthrosis / therapy
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Middle Aged
  • Netherlands / epidemiology
  • Outcome Assessment, Health Care
  • Prevalence
  • Quality of Life*
  • Surveys and Questionnaires
  • Young Adult
  • von Willebrand Diseases / complications*
  • von Willebrand Diseases / diagnosis
  • von Willebrand Diseases / drug therapy
  • von Willebrand Diseases / epidemiology*
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor