Comparing findings of routine Haemophilia Joint Health Score and Haemophlia Early Arthropathy Detection with UltraSound assessments in adults with haemophilia

Abstract

Joint bleeds in patients with haemophilia cause synovial hypertrophy and direct blood-related osteochondral changes. The ankles, knees and elbows are the most affected joints. To assess osteochondral changes in these joints, the radiologic Pettersson Score [1] and the clinical Gilbert Score [2] were developed. With the use of prophylactic replacement therapy the emphasis is on prevention of joint changes. As both scores are unable to detect early joint changes, the Gilbert score was further developed into the more sensitive Haemophilia Joint Health Score (HJHS) (2006) [3] and haemophilia-specific ultrasound protocols were introduced to assess synovial hypertrophy and cartilage defects. The 'Haemophlia Early Arthropathy Detection with UltraSound' (HEAD-US) protocol has been developed for non-radiologists, enabling direct assessment of joint health in conjunction with history taking and physical examination [4].Discrepancies between clinical function and imaging of haemophilia arthropathy (HA) are well known. In children with moderate or severe haemophilia on prophylaxis, routine ultrasound examination according to the HEAD-US protocol has shown to be able to detect osteochondral changes in joints without abnormalities on the HJHS and to evaluate whether minimal findings might be caused by osteochondral changes [5]. Yet, in adults, the value of HEAD-US in addition to HJHS has not yet been reported. The aim of this study is to explore the added value of routine HEAD-US examination after HJHS examination in adults with haemophilia.A retrospective cohort study was conducted using medical health records of adult patients with haemophilia collected during multidisciplinary check-up visits at the van Creveldkliniek at University Medical Center Utrecht between June 2015 and September 2015. Patients were excluded if they were wheelchair dependent, had a joint or muscle bleed <6 weeks prior to the assessment or underwent orthopaedic surgery <24 months prior to the assessment. No restrictions were made for severity of haemophilia. The study was approved by the Institutional Review Board of the University Medical Center Utrecht, The Netherlands.During the check-ups, both elbows, knees and ankles were examined according to the HJHS version 2.1 by a single physiotherapist (MT) with extensive experience with the HJHS. For each joint swelling (0-3), duration of swelling (0-1), atrophy (0-2), crepitus (0-2), range of motion (ROM) (flexion 0-3; extension 0-3), strength (0-4) and pain (0-2) were evaluated, adding up to a score between 0 and 20 per joint. Higher scores indicate worse joint function. Directly after HJHS examination, HEAD-US assessment was performed by one medical doctor (WF) [4]. The operator was trained according to the formal HEAD-US courses, including one initial training and two advanced HEAD-US courses. The HEAD-US protocol allows for the assessment and scoring regarding the degree of synovial hypertrophy (0-2), cartilage damage (0-4) and abnormalities of subchondral bone (0-2). Possible scores range from 0 to 8...

Joint status in Spanish haemophilia B patients assessed using the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD‐US) score

“…The development of MSK‐US in this field and the HEAD‐US protocol and scoring system have provided a unique opportunity to screen the joint status of HB patients at national level, as well as to observe the effect of treatment modality and HB severity on the establishment of haemophilic arthropathy. A few studies have recently been published on the use of this score in the clinic, thus providing experiences on feasibility and usefulness in real life …”

“…recently been published on the use of this score in the clinic, thus providing experiences on feasibility and usefulness in real life. [30][31][32][33] To our knowledge, this is the first study to apply the HEAD-US Despite changing practices in HB treatment, a notably high proportion of the overall cohort has healthy joints (HEAD-US score 0-1) F I G U R E 3 HEAD-US score of each joint according to haemophilia severity in patients treated on demand (OD). Joint health worsened significantly in all cases as severity increased.…”

Joint health and treatment modalities in Nordic patients with moderate haemophilia A and B – The MoHem study

“…13 (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) .32 13 (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19) 14 (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18) .98…”

Age‐related severity and distribution of haemophilic arthropathy of the knee, ankle and elbow among Chinese patients with haemophilia

“…5 In recent years, the musculoskeletal ultrasound (MSKUS) has been applied in the evaluation of both soft tissue and osteochondral changes of HA and was recommended by the World Federation of Haemophilia (WFH) for its advantages, such as convenience, time-effectiveness, high resolution of soft tissue and blood flow detection. [7][8][9] The definitive investigation into the age-related severity and distribution of HA using the HEAD-US scoring system has not been reported in developing countries yet. The validity and reliability of this system in evaluating HA have been previously confirmed.…”

“…The validity and reliability of this system in evaluating HA have been previously confirmed. [7][8][9] The definitive investigation into the age-related severity and distribution of HA using the HEAD-US scoring system has not been reported in developing countries yet. Herein, we present the severity and distribution of HA by analysing the knee, ankle and elbow arthropathies in Chinese patients with haemophilia.…”