Clinical and ultrasound evaluation of patients with haemophilia on prophylaxis

Abstract

Haemophilia is characterised by articular bleeding, mainly in elbows, knees and ankles. Repeated bleeding episodes induce synovial hypertrophy and progressive osteochondral changes that may lead to degenerative arthropathy. This is the most common complication in patients with haemophilia, and it causes disability and loss of quality of life. [1][2][3] Primary prophylaxis is the current gold standard in haemophilia care for the prevention of bleeding and ensuing joint damage. Dosage and treatment regimens vary across countries. Due to the limited access to factor concentrates and financial resources in developing countries, many children and young adults with haemophilia are treated with secondary or tertiary prophylaxis. [4][5][6] According to evidence, many patients experience joint degenerative changes despite prophylaxis, which suggests that subclinical bleeding occurs even with this type of treatment. 7,8 Early detection of joint bleeding, whether symptomatic or subclinical, preferably during childhood, helps prevent joint deterioration

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.…”

Age‐specific incidence of joint disease in paediatric patients with haemophilia: A single‐centre real‐world outcome based on consecutive US examination

“…All data, including haemophilia type, severity of disease, year of birth, age at prophylaxis initiation, history of symptomatic joint bleeding, level of physical activity, replacement therapy (prophylaxis vs. on‐demand) and presence/history of inhibitors were collected from electronic medical records. Regarding physical activity, the types of sports in which each patient participates were categorized according to the guidelines of the National Hemophilia Foundation 15 . Considering the history of joint bleeding, only data on the symptomatic bleeding history was collected and analysed.…”

“…Many reports have indicated that regular assessment of joint health using US was essential for detecting early signs of joint disease and preventing arthropathy 8–10 . Moreover, several studies have evaluated joint health using US in children 11–15 …”