From the Guidelines
Non-traumatic hematoarthrosis is most commonly caused by coagulopathy, particularly hemophilia A, as evidenced by the recent study on liver-related aspects of valoctocogene roxaparvovec gene therapy for hemophilia A 1.
Causes of Non-Traumatic Hematoarthrosis
The causes of non-traumatic hematoarthrosis can be categorized into several groups, including:
- Coagulopathy: Hemophilia A and B, von Willebrand disease, and other inherited bleeding disorders can lead to joint bleeding, as noted in the study on liver-related aspects of valoctocogene roxaparvovec gene therapy for hemophilia A 1.
- Anticoagulant therapy: Medications like warfarin, heparin, or direct oral anticoagulants can precipitate hemarthrosis, especially when the therapeutic range is exceeded.
- Hematologic causes: Thrombocytopenia and platelet dysfunction disorders can also lead to joint bleeding.
- Inflammatory joint conditions: Rheumatoid arthritis, pigmented villonodular synovitis, and synovial hemangiomas can cause bleeding through increased vascularity and synovial inflammation.
- Neoplastic processes: Synovial sarcoma and metastatic disease may erode blood vessels, resulting in joint bleeding.
- Vascular malformations: Vascular malformations near or within joints can rupture and cause hemarthrosis, as seen in some patients with knee arthroplasties 1.
- Other causes: Scurvy (vitamin C deficiency), neuropathic arthropathy (Charcot joint), and haemochromatosis can also lead to non-traumatic hematoarthrosis, with haemochromatosis being associated with a younger age of onset and rapid progression to cartilage loss 1.
Importance of Prompt Diagnosis
Prompt diagnosis of the underlying cause of non-traumatic hematoarthrosis is essential for appropriate management and prevention of joint damage. The study on haemochromatosis highlights the importance of early diagnosis and treatment, as haemochromatosis arthropathy can significantly affect quality of life and does not respond to phlebotomy 1. Therefore, it is crucial to identify the underlying cause of non-traumatic hematoarthrosis and provide targeted treatment to prevent long-term joint damage and improve patient outcomes.
From the Research
Non-Traumatic Hematoarthrosis
- Non-traumatic hematoarthrosis is often seen in patients with hemophilia, a rare X-linked inherited bleeding disorder caused by a deficiency in coagulation factors VIII and IX 2.
- Recurrent joint bleeding (hemarthrosis) is a common clinical manifestation of severe hemophilia, and if left unmanaged, can lead to the development of hemophilic arthropathy, a disabling condition characterized by joint remodeling, chronic pain, and reduced quality of life 2, 3.
- Other potential causes of non-traumatic hematoarthrosis include acquired hemophilia, which can occur in patients with rheumatoid arthritis 4.
- Factor VIII deficiency is a key factor in the development of non-traumatic hematoarthrosis in patients with hemophilia A, and replacement therapy with factor VIII can help prevent joint bleeding and preserve joint function 2, 5.
Pathophysiology
- Joint bleeding in hemophilia induces synovial inflammation, cartilage degeneration, and bone damage, resulting in a vicious circle of joint damage and further bleeding 3.
- The inflamed synovium affects the cartilage, while cartilage is also directly affected by blood via the release of cytokines and metalloproteinases, and via hydroxyl radical formation inducing chondrocyte apoptosis 3.
- Iron plays a pivotal role in the pathogenesis of hemophilic arthropathy, as does the fibrinolytic system 3.
Management
- Prophylactic coagulation factor replacement therapy using extended half-life recombinant drugs has significantly improved patients' quality of life by reducing the burden of intravenous injections 2.
- Non-replacement therapies such as subcutaneous emicizumab injections have also improved treatment adherence and led to greater protection of patients with hemophilia A 2.
- Point-of-care ultrasound imaging has improved the diagnosis of acute hemarthrosis and early hemophilic arthropathy, and allowed for better monitoring of progressive joint damage 2.