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Review
. 2024 Nov;30(6):1265-1271.
doi: 10.1111/hae.15116. Epub 2024 Nov 4.

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

Cihan Ay  1 , Maria Elisa Mancuso  2   3 , Davide Matino  4 , Karen Strike  5 , Gianluigi Pasta  6
Affiliations
Review

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

Cihan Ay et al. Haemophilia. 2024 Nov.

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.

Keywords: clinical relevance; haemophilia; health; joint disease; joints.

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Conflict of interest statement

Cihan Ay received personal fees for lectures and/or participation in advisory boards from Bayer, Biomarin, Biotest, CSL Behring, Novo Nordisk, Pfizer, Roche, LFB, Takeda, and SOBI. Maria Elisa Mancuso has acted as paid consultant, speaker and or advisor for Bayer, Biomarin, CSL Behring, Kedrion, LFB, Novo Nordisk, Octapharma, Pfizer, Roche, Sanofi, Sobi, and Takeda. Davide Matino reports research grants paid directly to the Institution (McMaster University) from Bayer, Pfizer, Novo Nordisk, Sanofi, Spark, Octapharma, Roche; personal fees/honoraria from Sanofi, Sobi, Novo Nordisk, Bayer, Pfizer, Octapharma, and Roche for participation in advisory boards, lectures and preparation of educational material. Karen Strike received research support from Hamilton Health Sciences Health Professional Clinical Research Award, Health Professional Investigator Award, McMaster Children's Hospital Foundation, Pfizer Canada, and Bayer; travel support from Pfizer, Bayer, Novo Nordisk; and consultation fees from Sanofi, Bayer, Hemalytic, Takeda, Pfizer, Biogen, Novo Nordisk, Roche, Baxalta, and Octapharma. Gianluigi Pasta received reimbursement for attending symposia/congresses and/or honoraria for speaking and/or consulting from Bayer, Kedrion, Novo Nordisk, Octapharma, Pfizer, Roche, Sobi, and Takeda.

Figures

FIGURE 1
FIGURE 1
PRISMA flow diagram of the literature search performed for this review. A literature search was conducted on papers published between 2011 and 2023 that used the Haemophilia Joint Health Score (HJHS) in the context of haemophilia.
See this image and copyright information in PMC

References

    1. Feldman BM, Funk SM, Bergstrom BM, et al. Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: validity of the hemophilia joint health score. Arthritis Care Res (Hoboken). 2011;63(2):223‐230. - PubMed
    1. 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. - PMC - PubMed
    1. Gouw SC, Timmer MA, Srivastava A, et al. Measurement of joint health in persons with haemophilia: a systematic review of the measurement properties of haemophilia‐specific instruments. Haemophilia. 2019;25(1):e1‐e10. - PMC - PubMed
    1. Hilliard P, Funk S, Zourikian N, et al. Hemophilia joint health score reliability study. Haemophilia. 2006;12(5):518‐525. - PubMed
    1. Feldman BM, Funk S, Hilliard P, et al. Hemophilia Joint Health Score (HJHS) 2.1. 2011; Accessed 15 January, 2024. https://elearning.wfh.org/resource/hemophilia‐joint‐health‐score‐hjhs/

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