Rapid musculoskeletal ultrasound for painful episodes in adult haemophilia patients

Abstract

Little objective information exists about musculoskeletal bleeding patterns in haemophilic arthropathy. Bleeding is assumed to be the cause of painful joints or muscles. Clotting factor treatment is provided empirically, but often does not alleviate pain. We hypothesized that perception of pain aetiology is unreliable, and introduced point-of-care high-resolution musculoskeletal ultrasound (MSKUS) to differentiate intra-articular bleeds vs. joint inflammation, and intra-muscle bleeds vs. other regional pain syndromes. To assess painful musculoskeletal episodes in adult haemophiliacs, we used rapid MSKUS, employing grey scale and power Doppler examination. Forty episodes in 30 adult haemophiliacs were evaluated. Thirty three of the 40 episodes were patient-reported as 'bleeding', five as 'arthritis-type' pain and two as 'undecided'. Of the 33 bleeding reports, only 12 were confirmed by MSKUS; the other episodes revealed other pathology. In contrast, three of five perceived arthritis flares were reclassified as bleeds. Similarly, physician assessment was incorrect in 18 of 40 instances. Swelling and warmth were present in approximately half of confirmed bleeding and non-bleeding episodes, and therefore not useful clinically. Few of the painful episodes were symptom controlled at the time of MSKUS. Management changed based on objective imaging findings in >70% of episodes, which resulted in symptom improvement >60% of the time. Significant discrepancies exist between MSKUS findings and patient/physician-perceived pain classification as bleeding or other musculoskeletal symptoms. Current practice of prescribing clotting factor or conservative measures based on pain perception seems inadequate and suggests that point-of-care imaging should be included into modern haemophilia care.

Recombinant long-acting glycoPEGylated factor IX in hemophilia B: a multinational randomized phase 3 trial

“…Another explanation could be that symptoms of hemophilic arthropathy, such as pain, swelling, and stiffness, may have been perceived as bleeding episodes, and therefore treated as such, even though no bleeding episode had actually occurred. 24 The results presented here demonstrate wide interpatient variability, and this unexpected finding requires further investigation.…”

Advances and challenges in hemophilic arthropathy

“…This prompted investigation into alternative new imaging modalities, which led to the adoption of musculoskeletal ultrasound (MSKUS) for the imaging of hemophilic joints and its recent introduction into clinical practice. 25–28 Continued innovative development and wide-spread utilization for HA care are expected over the next few years. Advances in ultrasound technology permit high resolution imaging for detailed visualization of anatomy and pathology of the musculoskeletal system, including tendons, muscles, ligaments and fluid.…”

“…MSKUS examination with sonopalpation is able to distinguish complex bloody from simple serous effusions with high accuracy as confirmed by needle aspiration. 25, 35 In addition, power Doppler permits rapid and dynamic assessment of synovial blood flow, thereby demarcating synovitis from fluid. In contrast, conventional MRI cannot easily differentiate joint fluid from synovitis without contrast administration, 36 nor can it easily distinguish bloody from non-bloody effusions (Figure 2).…”

“…The ability to diagnose inflammatory soft tissue changes and synovitis with power Doppler 25, 2633, 35 is important in HA. As in rheumatoid arthritis (RA), synovial hypertrophy is often present and can be accompanied by synovitis characterized by increased synovial blood flow.…”

Orthopaedics: Joint effort

“…Von Drygalski's research shows that only one-third of painful episodes reported by people with haemophilia are associated with bleeding into the joint 1 . Similarly, physicians find it hard to determine the cause of joint pain: in one small study 1 , von Drygalski and her colleagues found that physicians' assessments, based on patient interviews and physical examinations, were incorrect in 18 of 40 instances.…”