Long-term impact of joint bleeds in von Willebrand disease: a nested case-control study
Karin P.M. van Galen,
Piet de Kleijn,
Wouter Foppen,
Jeroen Eikenboom,
Karina Meijer,
Roger E.G. Schutgens,
Kathelijn Fischer,
Marjon H. Cnossen,
Joke de Meris,
Karin Fijnvandraat,
Johanna G. van der Bom,
Britta A.P. Laros-van Gorkom,
Frank W.G. Leebeek,
Eveline P. Mauser-Bunschoten
Affiliations
Karin P.M. van Galen
Van Creveldkliniek, University Medical Center Utrecht, the Netherlands
Piet de Kleijn
Van Creveldkliniek and Department of Rehabilitation, Physical Therapy Science and Sports, University Medical Centre Utrecht, the Netherlands
Wouter Foppen
Department of Radiology, University Medical Center Utrecht, the Netherlands
Jeroen Eikenboom
Department of Thrombosis and Hemostasis and Einthoven Laboratory for Vascular and Regenerative Medicine, Leiden University Medical Center, the Netherlands
Karina Meijer
Department of Hematology, University of Groningen, University Medical Center Groningen, the Netherlands
Roger E.G. Schutgens
Van Creveldkliniek, University Medical Center Utrecht, the Netherlands
Kathelijn Fischer
Van Creveldkliniek and Julius Center Department of Epidemiology, University Medical Center Utrecht, the Netherlands
Marjon H. Cnossen
Department of Pediatric Hematology, Erasmus University Medical Center-Sophia Children’s Hospital, Rotterdam, the Netherlands
Joke de Meris
Dutch Society of Haemophilia Patients, Leiden, the Netherlands
Karin Fijnvandraat
Department of Pediatric Hematology, Academisch Medisch Centrum, Emma Children’s Hospital, Amsterdam, the Netherlands
Johanna G. van der Bom
Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, and Department of Clinical Epidemiology, Leiden University Medical Center, the Netherlands
Britta A.P. Laros-van Gorkom
Department of Hematology, Radboud University Medical Center, Nijmegen, the Netherlands
Frank W.G. Leebeek
Department of Hematology, Erasmus University Medical Center, Rotterdam, the Netherlands
Eveline P. Mauser-Bunschoten
Van Creveldkliniek, University Medical Center Utrecht, the Netherlands
Patients with severe von Willebrand disease (VWD) may develop arthropathy after joint bleeds. Information on its prevalence and severity is limited. We aimed to assess the occurrence and severity of arthropathy in VWD and its impact on daily life. 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. 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). Arthropathy was defined as a Hemophilia Joint Health Score of 10 or higher, or a Pettersson score over 3 of at least one joint. 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 occurred in 40% of the cases versus 10% of the controls (P
