![]() X-rays are widely available and may capture advanced joint changes but are insensitive to early change and unreliable for cartilage and soft tissue evaluation 1. X-ray grading systems/scores include the Arnold–Hilgartner score (progressive, soft tissue assessment included) 22 and the World Federation of Hemophilia–recommended and widely used Pettersson score (additive, soft tissue excluded) 23. A gap continues to exist in our ability to detect and impact this sequela at the preclinical or asymptomatic phase when the disease process is early and potentially reversible.Įvaluation of HA is a dynamic, multi-dimensional process which employs clinical outcome assessment tools, including the World Federation of Hemophilia Physical Examination Score (Gilbert score), the hemophilia joint health score (HJHS) 17– 19, and a number of imaging modalities 20, 21. HA remains a critical focus of research and study as this debilitating disease negatively impacts quality of life, physical activity, and bone health of the aging PwH population, whose life span is now comparable to that of the normal population 3, 4, 14– 16. However, the pathophysiology of HA remains incompletely understood, and the pathways are yet to be fully elucidated. There is evidence that the key processes involved in the pathogenesis of HA include intra-articular inflammation, mediated by hemoglobin release, iron deposition, cytokines, hydroxyl radicals, and the “inflammasome”, and neo-angiogenesis, mediated by pro-angiogenic factors such as vascular endothelial growth factor 12, 13. The musculoskeletal consequences of hemophilia constitute the largest cause of morbidity in persons with hemophilia (PwH) 2 musculoskeletal disease may be minimized by early initiation of prophylaxis yet has continued to occur 3– 9, and a single episode of hemarthrosis can impact long-term joint outcomes 10, 11. Spontaneous hemarthrosis is the hallmark of severe hemophilia, and hemophilic arthropathy (HA), characterized by synovial hypertrophy and cartilage and bony destruction, which ultimately results from repeated joint bleeding episodes 1. The disease is classified, on the basis of residual coagulation factor activity level, as severe (<1%), moderate (1–5%), or mild (5–40%). Hemophilia A and B are X-linked congenital bleeding disorders characterized by reduced or absent levels of coagulation factors VIII or IX, respectively. The review also raises unanswered questions, highlights the need for consolidated research efforts, and delineates future directions expected to advance this technology and optimize its use in this patient population. ![]() This review summarizes currently available data on the emerging role of this new imaging modality, its limitations, and gaps in knowledge. Musculoskeletal ultrasound-particularly, point-of-care musculoskeletal ultrasound-has emerged as a promising imaging modality for the early detection and management of hemophilic arthropathy, and for the evaluation of hemarthrosis and painful musculoskeletal episodes in patients with hemophilia. Hemophilic arthropathy assessment is a continually evolving process and is particularly challenging in children and young adults in whom joint disease may be missed or underestimated as obtaining serial “baseline” magnetic resonance imaging scans of multiple clinically asymptomatic or nearly asymptomatic joints may be unjustifiable and cost-ineffective. It results from repeated bleeding episodes in the joint and is characterized by synovial hypertrophy and cartilage and bony destruction. Bleeding with resultant hemophilic arthropathy constitutes the largest cause of morbidity in patients with hemophilia.
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