PB0663 - 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

Monday, July 11, 2022

6:30 PM – 7:30 PM

Location: Exhibition

Presentation Format: In-Person

Presenting Author(s)

Roberta Gualtierotti, MD, PhD

Assistant Professor
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Centro Emofilia e Trombosi Angelo Bianchi Bonomi
Milan, Lombardia, Italy

Co-Author(s)

AT

Addolorata Truma

Resident in Internal Medicine
Department of Pathophysiology and Transplantation, University of Milan
Milan, Lombardia, Italy
VB

Valentina Begnozzi

Physioterapist
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milano, Lombardia, Italy
Eugenia Biguzzi, MD

Hematologist
Hematology clinic, Oncology Institute of Southern Switzerland, EOC
Bellinzona, Ticino, Switzerland
SS

Simona Maria Siboni

Hematologist
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center
Milan, Lombardia, Italy
SA

Sara Arcudi, MD

Academic researcher, MD, PhD
Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico, Centro Emofilia e Trombosi Angelo Bianchi Bonomi
Milan, Lombardia, Italy
AC

Alessandro Ciavarella

PhD Student
Università degli Studi di Milano, Department of Biomedical Sciences for Health
Milan, Lombardia, Italy
EB

Elena Boccalandro

Physiotherapist
Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy
Milan, Lombardia, Italy
Flora Peyvandi, MD, PhD

Professor of Internal Medicine; Director of the Angelo Bianchi Bonomi Hemophilia & Thrombosis Center
Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
Milan, Lombardia, Italy

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.