Assessment of haemophilic joints in a low-resourced area using clinical tools: The effect of different types of prophylaxis.

Abstract

Introduction: Haemophilic arthropathy (HA) is the most frequent complication in people with haemophilia (PWH). MRI is the gold standard to assess HA, however, there are limitations to its use in low-resourced areas.

Aim: Primary; to compare clinical-functional, laboratory, and ultrasonographic joint scores with MRI scores to determine a reasonable alternative to MRI. Secondary; to identify the effect of various replacement therapies on the degree of joint involvement.

Material and method: Fifty PWH with at least one affected joint, with or without inhibitors, and receiving either on-demand treatment or secondary prophylaxis, were included. All participants had a joint assessment by clinical HJHS 2.1, functional FISH, HEAD-US, and MRI DENVER scores. Also, serum COMP level was assessed by ELISA for the PWH and 50 healthy subjects as control.

Results: The HJHS 2.1 scores had a significant positive correlation with HEAD-US and the MRI DENVER scores. The FISH score had a significant negative correlation with HJHS 2.1, HEAD-US, and MRI DENVER Scores. The serum COMP level was comparable between the PWH and the controls. The HEAD-US score had a significant positive correlation with the MRI score. All of the joints' scores for the PWH on Emicizumab prophylaxis showed significantly lower HJHS 2.1 and MRI DENVER scores but higher FISH score than the joint scores of the patients receiving other types of prophylaxis.

Conclusion: The clinical-functional joints assessment scores (HJHS 2.1, FISH) were objective tools that correlated significantly with the HEAD-US and the Denver MRI scores. Emicizumab prophylaxis led to better joint status.