The haemophilia joint health score for the assessment of joint health in patients with haemophilia.

Abstract

Introduction: The haemophilia joint health score (HJHS) is a tool used to assess joint changes in patients with haemophilia. There is lack of consensus on the interpretation of HJHS scores and their clinical relevance.

Aim: To evaluate available literature reporting HJHS changes over time and assess a possible cut-off value for clinically relevant outcomes and the ideal follow-up for a meaningful score change.

Methods: We conducted a literature search of studies published between 2011 and 2023 where the HJHS version 2.1 had been adopted to detect changes in joint health in patients with haemophilia. We focused on studies that assessed clinical relevance of HJHS changes, evaluated the use of cut-off values and reported a follow-up over time.

Results: Our search identified 213 publications of which 53 (25%) were deemed relevant for this review. Of these, 33 (62%) publications reported the total HJHS score and 20 (38%) reported a single joint HJHS score, while the way of reporting HJHS scores/change was highly variable. Ten publications (19%) assessed clinical relevance, but their methods of calculation differed (defining a cut-off score, measuring standardised response mean or minimal detectable change). The follow-up duration varied from 2 weeks to 8 years in these 10 studies.

Conclusions: High variability in assessing HJHS change over time is the primary consequence of its low sensitivity, and the lack of consensus on interpretation and clinical relevance of the score. Therefore, more sensitive tools should be used alongside HJHS to better define the joint health status of patients with haemophilia.