Joint disease in haemophilia: Pathophysiology, pain and imaging.

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Abstract

[Indexed for MEDLINE] 4. Haemophilia. 2024 Apr;30 Suppl 3:120-127. doi: 10.1111/hae.15004. Epub 2024 Apr 5. Haemophilic arthropathy: Diagnosis, management, and aging patient considerations. Bakeer N(1)(2), Saied W(3), Gavrilovski A(4), Bailey C(5). Author information: (1)Indiana Haemophilia and Thrombosis Centre, Indianapolis, Indiana, USA. (2)Children's Hospital of Philadelphia, Pennsylvania, USA. (3)Department of Paediatric Orthopaedic Surgery, Children's Hospital "Bechir HAMZA", Medical School of Tunis, University Tunis-El Manar, Tunis, Tunisia. (4)University Clinic for Orthopaedic Surgery-TOARILUC, Clinical Centre Mother Theresa Skopje, Skopje, North Macedonia. (5)Orthopaedic Haemophilia Treatment Centre at Orthopaedic Institute for Children Los Angeles, Los Angeles, California, USA. Gene therapy and universal use of safer, more effective, and personalised prophylactic regimens (factor, and nonfactor) are expected to prevent joint bleeding and promote joint health in persons with haemophilia (PwH). Growing evidence suggests that subclinical bleeding, with active and inactive synovial proliferation, continues and haemophilic arthropathy remains a major morbidity in PwH despite early institution of joint prophylaxis. Joint health assessment is evolving with physical examination scores complementing imaging scores. Point-of-care ultrasound is emerging as a safe, cost-effective, and readily available tool for acute determination of musculoskeletal abnormalities, serial evaluation of joints for sonographic markers of haemophilic arthropathy, and in providing objective insight into the efficacy of new therapies. In acute haemarthrosis, arthrocentesis expedites recovery and prevent the vicious cycle of bleed-synovitis-rebleed. When synovial proliferation develops, a multidisciplinary team approach is critical with haematology, orthopaedics, and physiotherapy involvement. Synovectomy is considered for patients with chronic synovitis that fail conservative management. Non-surgical and minimally invasive procedures should always be offered and considered first. Careful patient selection, screening and early intervention increase the success of these interventions in reducing bleeding, pain, and improving joint function and quality of life. Chemical synovectomy is practical in developing countries, but radioactive synovectomy appears to be more effective. When surgical synovectomy is considered, arthroscopic/minimally invasive approach should be attempted first. In advanced haemophilic arthropathy, joint replacement and arthrodesis can be considered. While excited about the future of haemophilia management, navigating musculoskeletal challenges in the aging haemophilia population is equally important. © 2024 John Wiley & Sons Ltd. DOI: 10.1111/hae.15004