Ultrasound evaluation of joint damage and disease activity in adult patients with severe haemophilia A using the HEAD‐US system
- 23 February 2021
- journal article
- research article
- Published by Wiley in Haemophilia
- Vol. 27 (3), 479-487
- https://doi.org/10.1111/hae.14280
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.Keywords
Funding Information
- Pfizer
This publication has 36 references indexed in Scilit:
- Controlled, cross‐sectional MRI evaluation of joint status in severe haemophilia A patients treated with prophylaxis vs. on demandHaemophilia, 2014
- Definitions in hemophilia: communication from the SSC of the ISTHJournal of Thrombosis and Haemostasis, 2014
- A systematic review of MR imaging as a tool for evaluating haemophilic arthropathy in childrenHaemophilia, 2013
- Development and definition of a simplified scanning procedure and scoring method for Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)Thrombosis and Haemostasis, 2013
- Guidelines for the management of hemophiliaHaemophilia, 2012
- Past, present and future of hemophilia: a narrative reviewOrphanet Journal of Rare Diseases, 2012
- Ultrasound detects joint damage and bleeding in haemophilic arthropathy: a proposal of a scoreHaemophilia, 2010
- Comparison of radiography, CT and MR imaging in detection of arthropathies in patients with haemophiliaHaemophilia, 2009
- Prophylaxis versus Episodic Treatment to Prevent Joint Disease in Boys with Severe HemophiliaThe New England Journal of Medicine, 2007
- The Hemophilias — From Royal Genes to Gene TherapyThe New England Journal of Medicine, 2001