Consistency of Serial Ultrasonographic Joint Tissue Measurements by the Joint tissueActivity and Damage Exam (JADE) protocol in Relation to Hemophilic Joint Health Parameters

Abstract

Objectives The Joint tissueActivity and Damage Exam (JADE) is a point-of-care (POC) musculoskeletal ultrasound (MSKUS) protocol for non-radiologists to evaluate hemophilic arthopathy. Our aim was to determine the consistency of cross-sectional analyses of direct tissue measurements (JADE protocol) and clinical and functional joint assessments at three clinic visits. Methods We prospectively studied adults (n = 44) with hemophilia (A or B) of any severity and arthropathy at 3 North American sites. We assessed Hemophilia Joint Health Score (HJHS), total arc, and JADE parameters (bilateral elbows, ankles, and knees) at study entry, at ~ 12–18 months, and at ~ 24–36 months. JADE measurements included osteochondral alterations, cartilage thickness, and soft tissue expansion at sentinel positions. Associations between joint HJHS and total arc with each JADE variable were examined with random intercept models. Results At each visit increasing HJHS and decreasing total arc were associated in the expected direction with increasing length of OAs and soft tissue expansion in all joints, and decreasing cartilage thickness in the knee. However, HJHS associations with cartilage thickness were U-shaped for elbow and ankle (i.e. cartilage thinning and thickening). Associations between total arc and cartilage thickness followed a similar curve. (Near)normal levels of both joint parameters (HJHS and total arc) were associated with normal ranges of cartilage thickness. Conclusions POC MSKUS applying direct tissue measurements using the JADE protocol provided reproducible cross-sectional associations with joint health outcomes on three visits. These findings advance protocol validation and enable iterative adaptations resulting in JADE protocol version 2.

Haemophilic arthropathy: Diagnosis, management, and aging patient considerations

“…US detected more early joint changes (synovitis and osteochondral damage), while HJHS 2.1 showed the added value of detecting relevant physical and functional changes. [23][24][25][26][27] Another recent systematic review and meta-analysis included fifteen studies reporting on swelling at physical examination and synovial proliferation on US in 2890 joints of 627 patients concluded that joint swelling had low sensitivity for presence of US-detected synovial proliferation in haemophilia, suggesting underestimation of synovial proliferation by physical examination alone. 28 Prevalence of subclinical synovial proliferation in the reviewed studies ranged from 0% to 55%.…”

“…POC-MSKUS is a safe, cost-effective, readily available, and sensitive tool that allows for accurate diagnosis of acute haemarthrosis 19,26,33,34 and serial evalua-tion of synovial proliferation (active vs. inactive) 18,28,29 and the overall condition of osteochondral surfaces of index joints in PwH. 22,26 It is crucial to point out that while early diagnosis and management of haemophilic arthropathy is becoming more relevant for clinical management and individualised patient regimens 29 evidence to conclude that US-detectable findings are sensitive to or could inform changes or intensification of prophylactic therapy in PwH remains insufficient. 23 Significant gaps in the evidence for all assessment tools remain, and these tools complement one another in painting an accurate picture of the joint status.…”

“…Compared to clinical assessment, US has good correlation with HJHS 2.1 scores suggesting that they both provide complementary data on joint disease in adults and children with haemophilia; US detected more early joint changes (synovitis and osteochondral damage), while HJHS 2.1 showed the added value of detecting relevant physical and functional changes 23–27 28 .…”