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Determinants of impaired quality of life in patients with fibrous dysplasia.

Abstract：

BACKGROUND:Fibrous dysplasia is a rare bone disorder, commonly associated with pain, deformity and fractures, which may significantly impact on quality of life. In this study we evaluate quality of life in patients with fibrous dysplasia using the Short Form-36 and the Brief Pain Inventory questionnaires. Data were compared with those of the general Dutch population. RESULTS:Out of 138 patients from a cohort of 255 patients with fibrous dysplasia that were sent questionnaires assessing quality of life and pain, the response rate was 70.3%, with 97 patients, predominantly female (65%), completing the questionnaires. Monostotic fibrous dysplasia was predominant (n = 62, 64%). Fibrous dysplasia patients had significantly lower quality of life outcome scores than the general Dutch population for all tested domains of the Short Form-36 except for the "Mental health" and the "Role emotional" domains. More severe forms of fibrous dysplasia, had the more severe Short-Form-36 quality of life outcomes, but there was no significant difference in Brief Pain Inventory domains between different subtypes of fibrous dysplasia. Quality of life was lower in patients with higher disease burden, as reflected by high skeletal burden scores (p = 0.003) and high levels of P1NP (p = 0.002). CONCLUSION:We demonstrate impairments in all domains of quality of life, except for 'Mental health' and 'Role emotional' domains, across the wide spectrum of fibrous dysplasia including its milder forms. We identified high skeletal burden scores, reflecting disease severity, as the most consistent predictor of impaired quality of life. Our findings hold significant clinical implications as they draw attention to the clinically unmet need to address quality of life issues in the management of patients with all subtypes of fibrous dysplasia, including its milder forms.

journal_name

Orphanet J Rare Dis

journal_title

Orphanet journal of rare diseases

authors

Majoor BCJ,Andela CD,Bruggemann J,van de Sande MAJ,Kaptein AA,Hamdy NAT,Dijkstra PDS,Appelman-Dijkstra NM

doi

10.1186/s13023-017-0629-x

subject

Has Abstract

pub_date

2017-04-27 00:00:00

pages

issue

issn

1750-1172

pii

10.1186/s13023-017-0629-x

journal_volume

pub_type

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Determinants of impaired quality of life in patients with fibrous dysplasia.