Assessment of Annual Rate Changes of Intraarticular Tissue Measurements in Hemophilic Joints Experiencing Acute Bleeding Using the Joint Activity Damage Exam (JADE) Ultrasound Protocol

Background: The Joint Activity and Damage Exam (JADE) ultrasound protocol has been validated to measure key joint tissues (cartilage thickness, osteochondral alterations, and soft tissue expansion) at sentinel positions in patients with hemophilia. The effect of an acute joint bleed on longitudinal rate changes in these tissues has not been established.

Aims: Determine the effect of acute joint bleeding on annual rate changes of JADE measurements.

Methods: We prospectively evaluated 44 adult patients with hemophilia (A or B) of any severity at three Hemophilia Treatment Centers in the United States. Inclusion criteria required at least one arthropathic joint with Hemophilia Joint Health Score (HJHS) >3. JADE measurements and HJHSs were captured at 3 times points (study entry, ≈12-18 months, and ≈24-36 months). We calculated rates of change in JADE measurements in these joints. Rates of change of joint tissue that experienced an acute bleed confirmed by musculoskeletal ultrasound during the study period were compared to those that did not experience an acute bleed.

Results: Most patients in the cohort had severe hemophilia A and were on regular Factor VIII prophylaxis. The median age was 36 [IQR 28,49] years. A total of 264 joints were evaluated longitudinally. Of these joints, 248 (94%) experienced no acute joint bleeds over the study period. While the annual bleed rate was low (<1), 16 joints (4 elbows, 8 ankles, and 4 knees) experienced at least one acute joint bleed. HJHSs remained stable and there was no difference in rate changes of JADE measurements in bleeding joints when compared to non-bleeding joints.

Conclusions: These data suggest that rare joint bleeds may not affect the trajectory of joint health in adult patients who are otherwise well controlled on prophylaxis.