Joint health status in people with moderate hemophilia A: a cross-sectional multicenter study

Abstract

Background: The prevalence of arthropathy in people with moderate hemophilia A (mHA) is highly variable. People with mHA are often undertreated, and this may lead to joint damage and worsen their quality of life. Objectives: The aim of the present study was to evaluate joint status in mHA by means of point-of-care ultrasound (PoCUS) and clinical examination. Methods: Consecutive people with mHA receiving on-demand replacement treatment underwent a clinical examination of joint status according to the Hemophilia Joint Health Score (HJHS) protocol. On the same day, all patients underwent a PoCUS assessment according to the Hemophilia Early Detection by UltraSound (HEAD-US) protocol. Results: A total of 51 subjects were included. The median HJHS score was 2.0 (IQR, 0-3.0). A 0 to 1 HJHS score was found in 23 people with mHA (45.1%), between 2 and 3 in 17 (33.3%) and >3 in 11 (21.6%). The median HEAD-US score was 2.0 (IQR, 1-7), and a statistically significant correlation between HJHS and HEAD-US was found (rho = 0.732; P < .001). Osteochondral damage was found in 21.6% of patients, and hypertrophic synovium (HS) was found in 29.4%. Among those reporting a 0 to 1 HJHS score, 13.0% showed HS. Analysis at the joint level showed that the most commonly affected joint was the ankle, both for osteochondral damage and the presence of HS. Conclusion: Our study suggests that the prevalence of arthropathy changes in people with mHA receiving on-demand treatment is not negligible and that PoCUS is able to detect osteochondral damage as well as HS in this clinical setting. A more extensive screening of the joint status could be useful to tailor treatment and improve outcomes in mHA.