title: The association of haemophilic arthropathy with Health-Related Quality of Life: a post hoc analysis.
abstract: BACKGROUND
  The aim of replacement therapy in haemophilia is to improve Health-Related Quality of Life (HRQoL) by preventing bleeding and arthropathy. However, the association of arthropathy with HRQoL is unknown.
  
  
  OBJECTIVE
  To explore the association of haemophilic arthropathy with HRQoL.
  
  
  METHODS
  A post hoc analysis on patients with severe/moderate haemophilia with SF36 questionnaire (SF36) and X-rays of ankles, knees and elbows made within 2.5-years. The SF36 scores of 'physical functioning' (SF36-PF, range 0-100, optimum 100) and Utility (SF6D-Utility, range 0-1, optimum 1) and radiological Pettersson scores (PS, range 0-78, optimum 0) were calculated. The association of PS with reduced SF6D-Utility and SF36-PF (<age-specific normal values) was analysed using ROC analyses and multivariable logistic regression.
  
  
  RESULTS
  Overall, 176 assessments were analysed: 130 from the Van Creveldkliniek and 46 from a French multicenter study. Most patients had severe haemophilia (89.9%), evaluated at 26.6 years, and with a range 15.7-65.8. Overall PS median (interquartile range) was 16 (7-34), SF6D-Utility was 0.76 (0.64-0.86) and SF36-PF was 85 (60-95). Receiver operating curve analysis identified a threshold PS of 21 points for both SF6D-Utility (AUC 0.65) and SF36-PF (AUC 0.76). In patients with PS > 21 points, the risk of reduced SF6D-Utility was stable (OR 4.16; 95% CI: 2.03-8.51) but SF36-PF continued to decrease: compared to lowest PS, OR for reduced SF36-PF was 5.69 (95% CI: 1.62-20.06) for PS 22-39 and 25.15 (95% CI: 6.53-96.81) for PS 40-78.
  
  
  CONCLUSIONS
  Health-Related Quality of Life only showed a significant deterioration in patients with a Pettersson score of >21 points. This suggests that HRQoL is relatively insensitive to early joint changes.
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: K
authors: K
authors: Fischer
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: P
authors: P
authors: de Kleijn
authors: Department of Rehabilitation, Nursing Science and Sports, University Medical Center Utrecht, Utrecht, the Netherlands.|Unité d'Hémostase Clinique, Center Régional de traitement de l'Hémophilie, Lyon, France.
authors: C
authors: C
authors: Negrier
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: E P
authors: EP
authors: Mauser-Bunschoten
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: P R
authors: PR
authors: van der Valk
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: K P M
authors: KP
authors: van Galen
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: A
authors: A
authors: Willemze
authors: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors: R
authors: R
authors: Schutgens
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006467:Hemophilia A / Q000150:complications*; D006801:Humans; D007592:Joint Diseases / Q000150:complications*; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D016896:Treatment Outcome; D055815:Young Adult
keywords: Quality of Life; arthropathy; haemarthrosis; radiograph; utility