Development and definition of a simplified scanning procedure and scoring method for Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US)

Abstract

The aim of this study was to develop a simplified ultrasound scanning procedure and scoring method, named Haemophilia Early Arthropathy Detection with UltraSound [HEAD-US], to evaluate joints of patients with haemophilic arthropathy. After an initial consensus-based process involving a multidisciplinary panel of experts, three comprehensive and evidence-based US scanning procedures to image the elbow, knee and ankle were established with the aim to increase sensitivity in detection of early signs of joint involvement while keeping the technique easy and quick to perform. Each procedure included systematic evaluation of synovial recesses and selection of a single osteochondral surface for damage analysis. Based on expert consensus, a simplified scoring system based on an additive scale was created to define the joint status and, in perspective, to offer a tool to evaluate disease progression and monitor the result of treatment in follow-up studies.

Evaluation of early musculoskeletal disease in patients with haemophilia

“…It is of note that some abnormalities are better detected with MRI, such as subchondral cysts, cartilage loss and haemosiderin deposits [25]. To improve consistency in determining joint status, simplified innovative protocols and scoring techniques have been developed for both ultrasound and MRI, that is, the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) and the compatible additive MRI scale, respectively [26,27]. In addition, X-rays scored using the Pettersson scale can still be informative for patients with haemophilia and established joint damage [28].…”

Ultrasound for Early Detection of Joint Disease in Patients with Hemophilic Arthropathy

“…Although the widespread use of prophylaxis has been able to significantly reduce the onset of arthropathy, it has been shown that a non-negligible percentage of patients develop degenerative changes in their joints despite this type of treatment [ 4 , 5 ]. With the aim to identify early arthropathic changes and prevent the development or progression of hemophilic arthropathy, periodic monitoring of the joint status in hemophilia patients has been recommended in the framework of comprehensive care [ 9 , 10 ]. Considering that the sensitivity and specificity of physical examination assessment scores (e.g., Gilbert Orthopedic Joint Score—WFH—and the Hemophilia Joint Health Score—HJHS) remain challenging in the identification of early, subclinical joint abnormalities, and that the severity of joint impairment could be missed [ 5 , 11 , 12 , 13 ], the use of radiography and magnetic resonance (MR) imaging has been recommended as a complement to clinical examination for assessing the joint status and following the disease progression in hemophilia patients [ 14 ].…”

The Progression of Hemophilic Arthropathy: The Role of Biomarkers

“…Contradictory results are reported in the literature on the ability of US to detect intra-articular deposition of hemosiderin. Some authors described some distinctive echo textural features between hemosiderin and synovium [ 41 , 49 , 53 , 54 ] but other authors did not find any difference between the US appearance of hemosiderin-laden and hemosiderin-free synovium [ 55 , 56 , 57 ].…”