Assessment of Hemophilic Arthropathy by Ultrasound: Where Do We Stand?

Abstract

Joint hemorrhages represent the most common type of bleeding episode in persons with hemophilia, and recurrent hemarthrosis triggers chronic arthropathy, which is the most frequent chronic complication in these patients. In recent years, in the frame of a comprehensive care approach, a growing attention has been given to the periodic assessment of the joint status in hemophilia patients with the aim to identify early arthropathic changes and to prevent the development of a clinically overt arthropathy. Besides clinical examination, X-ray and magnetic resonance imaging (MRI) are currently used to evaluate joint status and to monitor the disease progression in hemophilia. Considering the limitations of X-ray and MRI, growing interest has been given to ultrasound (US) as a possible tool to assess joint status and identify early arthropathic changes in hemophilia patients. In the present review, we summarize major literature evidence on the use of joint US for the evaluation of markers of disease activity (joint effusion and synovial hypertrophy) and of degenerative damages (osteochondral changes) in patients with hemophilia. On the whole, being able to identify the presence of intra- or extra-articular fluid, US examination is the fastest and most reliable technique to identify acute conditions, such as hemarthrosis. In addition, the information on joint involvement provided by US in the patient follow-up may influence treatment decisions on a personalized basis. The use of US as part of a routine clinical examination by hemophilia experts may optimize the diagnostic workflow, avoiding additional costs and long waiting lists for patients referred to imaging departments. In the frame of a comprehensive care approach, US might represent a strategy to early detect and monitor synovial hypertrophy and osteochondral changes in hemophilia, thus extending the clinical examination and helping identify joints to be studied with a second-level examination such as MRI.

Evaluation of early musculoskeletal disease in patients with haemophilia

“…In many countries with limited resources, patients may not undergo MRI because of a lack of availability. Ultrasound is less expensive than MRI, globally available and more convenient and can be used to detect and monitor early synovial hypertrophy and osteochondral changes [37]. However, ultrasound can be observer-dependent and cannot visualize deep structures, so is not ideal for detecting subchondral cysts, loss of cartilage or haemosiderin deposits [38].…”

Treatment and Prevention of Bleeds in Haemophilia Patients with Inhibitors to Factor VIII/IX

“…The issue of subclinical bleeds is complex, because besides the current inability to detect them simply, strategies for their treatment and prevention are lacking. In order to improve our knowledge of these aspects, joint ultrasonography appears to be a promising approach [90] and, in the future, the identification and validation of biochemical markers of progressing joint disease could be useful to guide treatment [91]. However, the ‘acceptable’ number of joint bleeds may differ among patients because of inter-individual variation in the inflammatory response to bleeding.…”

Use of ultrasound for evaluation of painful joint episodes perceived as haemarthrosis in adult patients with severe haemophilia

“…2 The use of US is still limited in haemophilia despite its ease of use and existing standardized criteria for fluid and blood detection in the joint. [3][4][5][6] Judicious management of blood products is of foremost importance, particularly in countries with limited resources. This is the case of the Reference Centre for the treatment of adults with Haemophilia at the University of the Republic Hospital, in Montevideo, Uruguay.…”