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Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score in patients with hemophilia: experience from a single comprehensive care center

R. Gualtierotti1, A. Truma2, V. Begnozzi3, E. Biguzzi3, S. Siboni4, S. Arcudi5, A. Ciavarella6, E. Boccalandro3, F. Peyvandi7

1Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy, Milan, Lombardia, Italy, 2Department of Pathophysiology and Transplantation, University of Milan, Milan, Lombardia, Italy, 3Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy, Milan, Lombardia, Italy, 4Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy, Milan, Lombardia, Italy, 5Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy, MILAN, Lombardia, Italy, 6Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center and Fondazione Luigi Villa, Milan, Italy, Milan, Lombardia, Italy, 7Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Università degli Studi di Milano, Department of Pathophysiology and Transplantation, and Fondazione Luigi Villa, Milan, Italy., Milan, Lombardia, Italy

Abstract Number: PB0663

Meeting: ISTH 2022 Congress

Theme: Hemophilia and Rare Bleeding Disorders » Hemophilia - Clinical

Background: Recurrent joint bleeding leads to chronic arthropathy. The hemophilia joint health score (HJHS) is used to assess the joint health based on physical examination. More recently, musculoskeletal ultrasound (MSKUS) assessment has been introduced in the clinical practice and the hemophilia early detection with ultrasound (HEAD-US) score has been proposed.

Aims: This study aims to evaluate the correlation between the HJHS and the HEAD-US in evaluating the joint status and stage of arthropathy of patients with hemophilia.

Methods: In a cross-sectional study we obtained the HJHS and the HEAD-US in 100 patients with hemophilia A and B with different severity. The HJHS was based on the physical examination of the six index joints (elbows, knees and ankles) and gait assessment by a trained physiotherapist and the HEAD-US was performed by an expert rheumatologist with experience in MSKUS in order to evaluate synovitis and osteochondral damage on the same joints. Spearman’s rho was used to assess the correlations, significance was set at a p < 0.0001 (**).

Results: The mean age was 41 ± 19 years. The median score of HJHS 2.1 was 11 (3.75-17.25), the median score of HEAD-US was 8.5 (2-17,25). Based on ultrasound examination, 44% of patients had at least one joint with synovitis (HEAD-US score ≥1). There was a good correlation between HEAD-US and HJHS score (rho=0.74**). Furthermore, we observed a moderate to strong correlation between the joint total score of the HJHS and the total synovitis, cartilage and bone damage domains of the HEAD-US score (0.55**, 0.78**, 0.72**, respectively). Notably, the HJHS total pain domain correlated weakly with the HEAD-US cartilage and bone domains (0.33** and 0.30** respectively).

Conclusion(s): Our results show a good correlation and a complementarity between HJHS and HEAD-US scores. The routine assessment of both tools helps clinicians define the stage of arthropathy and inform personalised management.

Image

Figure 1. Correlation between the HJHS total score and the HEAD-US total score in the study population -n=100-

To cite this abstract in AMA style:

Gualtierotti R, Truma A, Begnozzi V, Biguzzi E, Siboni S, Arcudi S, Ciavarella A, Boccalandro E, Peyvandi F. Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score in patients with hemophilia: experience from a single comprehensive care center [abstract]. https://abstracts.isth.org/abstract/hemophilia-joint-health-score-hjhs-and-hemophilia-early-arthropathy-detection-with-ultrasound-head-us-score-in-patients-with-hemophilia-experience-from-a-single-comprehensive-care-center/. Accessed September 24, 2023.

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ISTH Congress Abstracts - https://abstracts.isth.org/abstract/hemophilia-joint-health-score-hjhs-and-hemophilia-early-arthropathy-detection-with-ultrasound-head-us-score-in-patients-with-hemophilia-experience-from-a-single-comprehensive-care-center/

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