The Hemophilia Joint Health Score (HJHS) version 2.1 can be used to detect arthropathy in adult patients with hemophilia, according to a new study published in Research and Practice in Thrombosis and Haemostasis.

A previous study found that the evidence for the use of HJHS in pediatric and young adults was strong but its use in adults needed further investigation. In this study, the HJHS scores tended to increase with age and disease severity for patients.

The median total scores were 9.0 for 18- to 29-year-olds, 27.0 for 30- to 40-year-olds, 36.0 for 40- to 50-year-olds, and 37.0 for those above 50 years old. These values were much higher than healthy controls who had values of 2.0, 3.0, 3.0, and 8.0 in the respective matching age groups. Using a Mann-Whitney U test, healthy controls and patients with hemophilia were significantly different from each other in each age group with P <.001 for each age.


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The median HJHS scores increased with disease severity in all age groups of patients with hemophilia. The median values for mild, moderate, and severe hemophilia were 0.5, 9.0, and 13.0 for the 18 to 29 age group, 3.0, 27.5, and 31 for the 30 to 40 age group, 13.0, 29.5, and 41.5 for the 41 to 50 age group, and 18.0, 29.0, and 42.0 for the 50 and above age group.

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The HJHS scores correlated with other joint and pain assessments. A high correlation (0.95) was found between the HJHS and the World Federation of Hemophilia Orthopedic Joint Score (also known as the Gilbert Score). Moderate to modest correlations were observed between the HJHS and the Short-Form McGill Pain Questionnaire, the Functional Independence Score of Hemophilia, and the Hemophilia Activities List.

Through the analysis of healthy adult men in the study, normative adult reference values were obtained for HJHS scores. For mean ages of 50 or less, the 95th percentile included scores up to 12 and up to 19 for men above the age of 50 years.

“We have demonstrated excellent convergent and discriminant construct validity of the HJHS in the adult population,” the authors concluded. “The adult normative values obtained from this study will provide context on HJHS scores resulting specifically from bleed-related damage and not age-related joint impairment as is seen in healthy adults without hemophilia.”

A total of 192 patients with hemophilia along with 120 age-matched healthy adults were recruited for the study from clinical sites in Canada, Belgium, India, the UK, and the US. The patients with hemophilia were made up of 68% severe, 14% moderate, and 18% mild cases.

Reference

St-Louis J, Abad A, Funk S, et al. The Hemophilia Joint Health Score version 2.1 validation in adult patients study: a multicenter international study. Res Pract Thromb Haemost. 2022;6(2):e12690. doi:10.1002/rth2.12690