Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study

Abstract

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints. Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3 rd , 6 th , 9 th , and 12 th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results. Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12 th month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12 th month. Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

Screening for subclinical synovial proliferation in haemophilia: A systematic review and meta‐analysis comparing physical examination and ultrasound

“…The reasons for excluding studies based on full text are summarized in Supplement S2. Eventually, 15 studies 11,12,15,24,29,[38][39][40][41][42][43][44][45][46][47] were included in the systematic review. The 15 included studies reported on occurrence of swelling and synovial proliferation in 2890 joints of 627 patients.…”

“…Eleven out of 15 included studies were considered 'screening of index joints' studies 24,29,[38][39][40][41][43][44][45][46][47] and were thus included in a bivariate random-effects model to determine summary estimates for sensitivity and specificity. Subclinical synovial proliferation was found in 4.6-41.7% of the joints in these studies.…”

“…In addition, the majority of studies were not specifically designed to compare physical examination and ultrasound for detection of synovial proliferation specifically, resulting in potential selection and/or information bias in 9 of 15 studies. While 12 of 15 included studies reported on the correlation between HJHS and ultrasound 11,12,46,47,24,29,[39][40][41][42]44,45 and 7 of 10 aimed at investigating the correlation between HJHS and ultrasound, 11,29,39,40,44,45,47 only De la Corte-Rodriguez et al 29 aimed at comparing the separate items of the HEAD-US and HJHS.…”

The value of ultrasonography in detecting early arthropathic changes and contribution to the clinical approach in patients of hemophilia

“…13 Studies in the literature have shown a correlation between HJHS, a clinical assessment scale, and HEAD-US. [22][23][24] It is seen that articular symptoms in subclinical cases can be detected earlier by HEAD-US.…”

Clinical and radiological assessment of joints in people with haemophilia in Assam, Northeast India: a cross-sectional study

“…However, as well as there being difficulties in using MRI with children, it is expensive and there is limited availability in resource-constrained settings. Ultrasound is effective in assessing changes to soft tissue and cartilage in early arthropathy [16,17] and its use with the HEAD-US scoring system has shown similar sensitivity [19] . X-rays are not sensitive enough to detect early arthropathy but can show changes in the later stages when arthropathy is already established.…”