Quantitative measurements of haemophilic joint tissues by point‐of‐care musculoskeletal ultrasound: Associations with clinical and functional joint outcome parameters

Abstract

Background Painful arthropathy is a long‐term complication in patients with hemophilia (PWH), affecting mobility and quality of life. A major barrier for the appraisal of joint health is the absence of point‐of‐care (POC) imaging modalities to promptly identify and manage arthropathic changes. Accordingly, we developed the Joint tissue Activity and Damage Exam (JADE) POC musculoskeletal ultrasound (MSKUS) protocol. JADE is validated for haemophilic joint tissue recognition with high intra/inter‐rater and inter‐operator reliability. Aims Evaluate associations of JADE with clinical (Hemophilia Joint Health Score, [HJHS]) and functional (total arc [combined flexion and extension range of motion [ROM]]) parameters. Methodology In this multi‐centre prospective study, we recruited PWH A or B with at least one arthropathic joint. We evaluated joint health (both elbows, knees, and ankles) by comparing JADE measurements (soft tissue and cartilage thickness, and osteochondral alterations) with HJHS and total arc. Results Of 44 PWH, most had hemophilia A (35/44), were severe (36/44) and had a median age of 36 years. Increasing HJHSs and declining total arc, indicating worsening arthropathy, were associated with JADE measurements in the expected direction, including (1) increasing length of osteochondral alterations, (2) diminished cartilage thickness, and (3) greater soft tissue expansion. The ankles had the highest proportion of joints without measurable (missing) cartilage. In multivariable models MSKUS measurements explained 68% and 71% of the variation in HJHS and total arc respectively for the elbow, 55% and 29% respectively for the knee, and 50% and 73% for the ankle. Conclusions This study highlights the associations of direct intra‐articular ultrasonography measurements using the JADE protocol with clinical and functional parameters. Our findings underscore the clinical value of POC MSKUS using the JADE protocol as a complementary instrument for the diagnosis and management of haemophilic arthropathy.

The role of point-of-Care Musculoskeletal Ultrasound for Routine Joint evaluation and management in the Hemophilia Clinic - A Real World Experience

“…Ankles were most affected and treated with PT, followed by knees and elbows. Of interest, we reported previously that the number of joints without measurable cartilage in ankles was much higher compared to elbows and knees when applying JADE measurements to the same cohort of PwH [ 38 ].…”

“…The JADE protocol has been validated by Outcomes Measures in Rheumatology (OMERACT) guidelines [ 35 ] on the tissue discrimination level [ 35 ], and has demonstrated high intra/inter-rater and inter-operator reliability [ 29 , 36 , 37 ]. Also, the direct tissue measurements correlate well with clinical and functional parameters used to evaluate PwH [ 38 ].…”

How to assess, detect, and manage joint involvement in the era of transformational therapies: Role of point‐of‐care ultrasound

“…Denver Score 13 and International Prophylaxis Study Group 14 ) and ultrasound (e.g. Haemophilia Early Arthropathy Detection with Ultrasound [HEAD-US], 15 Joint Tissue Activity and Damage Exam [JADE], 16,17 and Universal Simplified Ultrasound (US-US) protocol 18 ); for activities and participation, the Haemophilia Activities List (HAL) 19 and the Functional Independence Score in Haemophilia (FISH). 20 Haemophilic arthropathy involves complex processes that need to be incorporated into assessments for joint health throughout the disease course of haemophilia.…”

“…Physical examination may detect significant joint changes, whereas ultrasound can identify abnormalities that may be missed by physical examination alone 66 . HJHS clinical measures also correlate with intra‐articular ultrasound measurements using JADE in patients with haemophilia 17 . A systematic review of 98 study reports found that the HJHS is the most extensively studied joint assessment tool and is used in clinical practice to assess children and young adults receiving intensive treatment 67 .…”

“…16 JADE correlates with HJHS and total joint arc. 17 Both HEAD-US and JADE are valid for assessing early and late stages of articular changes. 17,39 Although JADE is highly sensitive for the diagnosis of haemarthrosis, 16 the longitudinal assessment of arthropathic joint changes in the chronic setting requires further validation.…”