Ultrasound evaluation of joint damage and disease activity in adult patients with severe haemophilia A using the HEAD‐US system

Abstract

Introduction The Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) system and scoring scale has proven to be an accurate and time‐efficient imaging method for identifying joint damage in patients with haemophilia. Aim Observational, multicentre, cross‐sectional study conducted in 8 centres in Spain that assessed the joint status of adult patients with severe haemophilia A (SHA) using HEAD‐US. Methods Joint status of the elbow, knee and ankle was evaluated in adults with SHA receiving on‐demand (OD) treatment, or primary (PP), secondary (SP), tertiary (TP) or intermittent (IP) prophylaxis. Results Of the 95 patients enrolled, 87 received prophylaxis (6.3% PP, 38.9% SP, 43.2% TP and 3.2% IP). Mean age was 35.2 years, and 59% of patients had not undergone image testing in the last year. The HEAD‐US score was 0 in all joints in 6.3% of patients. The ankle was the most affected joint, regardless of treatment regimen. Patients receiving OD treatment, TP or IP had the overall worst scores, mainly in the ankles and elbows; a similar but milder profile was observed in patients on SP; and patients on PP had the best score in all joints. Conclusion Joint function may be effectively preserved in patients with SHA on PP, but OD treatment or later initiation of prophylaxis does not seem to prevent progression of arthropathy. Disease worsening was observed in patients OD, TP or IP, most often affecting ankles and elbows. Closer ultrasound imaging monitoring may improve management of these patients.

Clinical assessment and point of care ultrasonography: How to diagnose haemophilic synovitis

“…Our work shows that 44% of PWH showed synovial hypertrophy in at least one of their joints, with the rate higher in those on tertiary or on‐demand prophylaxis and lower in those on primary prophylaxis. These findings are aligned with those published by Jimenez‐Yuste et al 25,26 …”

“…This difference could be due to the fact that the elbow is a less weight‐bearing joint, where synovitis is lodged in the posterior recesses of the deep olecranon fossa or in the anterior recesses under a thick muscle mass. Furthermore, several studies have already shown that in haemophilia, the elbows are affected as much as the knees and ankles 25,26 . Therefore, physical and ultrasound examination protocols should also assess this joint.…”

“…Furthermore, several studies have already shown that in haemophilia, the elbows are affected as much as the knees and ankles. 25,26 Therefore, physical and ultrasound examination protocols should also assess this joint.…”

The factor VIII treatment history of non‐severe hemophilia A: COMMENT. Joint damage in adult patients with mild or moderate hemophilia A evaluated with the HEAD‐US system

“…Here we report unpublished findings of a 28-adult male patient subset with moderate (n = 14) and mild (n = 14) HA, with median age of 42.5 [IQR 32-60.5] years (40.5 [IQR 30.0-49.0] and 51.0 [IQR 34.0-63.0] years for moderate and mild, respectively) from a previously performed cross-sectional study to assess joint status in Spain. 6 Not surprisingly we found 19 patients non-subjected to any evaluation by imaging modalities over the last 12 months (85.7% mild; 53.8% moderate), and also a HEAD-US score of 0 in all joints in 5 (35.7%) and 3 (21.4%) patients with mild and moderate HA, respectively. However, we observed 8 patients with target joint in at least one joint, being the ankles the most affected ones (4 patients), and total knee replacement was present in 1 mild patient (RK) and 1 moderate patient (LK).…”

“…A substantial number of OD patients scored 0 for each evaluated joint (57.1% to 72.7%), which, as observed in severe HA patients, may implicate a significant association between arthropathy and treatment modality, with worse results in patients with OD treatments. 6,8 Severe synovitis was only observed for 1 LK and 1 LA in the OD population, although that patients on TP were poorly controlled prior to the establishment of the prophylactic regimen and had a joint status already compromised.…”