Abstract
BackgroundIntra-articular bleeds in patients with inherited bleeding disorders lead to active synovitis which may progress to a chronic state over time. We explored the diagnostic value of color Doppler ultrasound in detecting synovitis in boys with bleeding disorders.ResultsSixty boys with hemophilia and 3 boys with type 3 von Willebrand disease aged 5 to 18 years (median 12.3 years) were imaged by gray-scale and color Doppler ultrasound (US) in three centers (Beijing, China [n = 22], Guangzhou, China [n = 12] and Toronto, Canada [n = 29])) in this observational study. Images were independently reviewed by two radiologists blinded to clinical data using a subjective semi-quantitative scoring system and objective measurements of synovial thickness and vascularity. Inter-reader reliability for using subjective versus objective color Doppler US methods for assessing synovial vascularity was excellent for the subjective method and moderate/lower range of substantial for the objective method. Agreement between degree of vascularity on color Doppler and extent of synovial hypertrophy on gray-scale US was overall poor for Canada data and moderate for China data. Correlations between degree of vascularity on color Doppler and synovial hypertrophy on gray-scale US, and clinical constructs (total and itemized HJHS scores and total Pettersson X-ray scores) for assessment of blood-induced arthropathy were all poor.ConclusionColor Doppler US is a valuable scoring method for evaluating reactive synovitis in joints of subjects with inherited bleeding disorders and holds potential for assessing post-bleed reactive synovitis once further information on its association with timing of the joint bleed becomes available in the literature.
Highlights
Intra-articular bleeds in patients with inherited bleeding disorders lead to active synovitis which may progress to a chronic state over time
Full list of author information is available at the end of the article
A total of 63 children and adolescents with ages ranging from 5 to 18 years, median age 12.3 years agreed to participate in the original studies conducted at the three participating hemophilia treatment centers and were imaged by gray and color Doppler ultrasound, 34 in China
Summary
Intra-articular bleeds in patients with inherited bleeding disorders lead to active synovitis which may progress to a chronic state over time. Recurrent joint bleeding and subsequent development of joint damage are complications in persons with moderate or severe hemophilia, in whom hemarthroses account for 70–80% of all bleeding episodes [1]. Intra-articular bleeds lead initially to active synovitis, characterized by hypervascularization and inflammatory activity, and microscopic degenerative changes in the cartilage. There may be progression to a chronic state of synovitis, where macroscopic cartilage erosions and arthropathy may be observed [2, 3]. Due to the occurrence of subclinical bleeding, early pathological synovial changes may go unrecognized. Such subtle undiagnosed synovial abnormalities may lead to irreversible arthropathy later on [4]. Accurate monitoring of joint status is an essential part of hemophilia care in order to guide treatment decision-making
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