Evaluation of Joint Condition in Daily Practice in the Era of New Hemophilia Treatment
With the development of new treatment options for hemophilia, there has also been progress in methods for monitoring hemophilic arthropathy and identifying it in its early stages. In addition to classical X-rays, clinical scoring systems, and the gold standard of magnetic resonance imaging (MRI), the so-called POCUS – point-of-care ultrasound – has emerged as a quick and reliable method for assessing joint health. Potential biomarkers for osteochondral damage and synovial proliferation specific to hemophilia or for detecting joint bleeding are also being investigated, though their routine use is not anticipated in the near future.
How to break the vicious cycle?
Hemophilic arthropathy results from a vicious cycle of bleeding, joint damage, and chronic inflammation, making the joint more prone to bleeding. Early detection of reactive synovial hypertrophy allows for the implementation of preventive strategies that help reduce joint damage and functional limitations, thereby improving patients' quality of life. Traditional clinical approaches and laboratory tests, however, may not display the sensitivity and specificity needed for monitoring joint health with current modern hemophilia therapy.
Assessment of functional ability
In addition to physical examination, this includes the HJHS (Hemophilia Joint Health Score), which captures changes in joint functionality, the FISH (Functional Independence Score in Haemophilia), which assesses patient independence, and the HAL (Haemophilia Activities List), which identifies problematic activities. A questionnaire focused on quality of life is also included. Together, these tools provide a basic picture of the patient's joint status, which is further refined by imaging methods.
Imaging methods
The gold standard for imaging hemophilic joints is, of course, magnetic resonance imaging (MRI), which comprehensively images the joint, including the presence of hemosiderin and subchondral cysts. It can also be used to monitor response to treatment. Its disadvantages include the cost, the use of contrast material, and the waiting time. Therefore, it is difficult to use as preventive imaging for all major joints.
X-ray (RTG) examination remains an important part of imaging already damaged joints but cannot be used for early signs of hemophilic arthropathy.
Ultrasound (US) is a relatively inexpensive, reliable, quick, and non-invasive method for assessing joint changes in hemophilic patients that can be used by non-radiologists. It can image both chronic changes (osteochondral damage and synovial hypertrophy) and acute joint issues, such as hemarthrosis. Additionally, synovial proliferation can indicate subclinical bleeding, enabling the examining physician to detect joint changes even in asymptomatic “non-bleeding” patients. The specificity and sensitivity of US hemophilic protocols are not far behind those of MRI. However, the yield of US is limited by the physician's experience and patient variability.
Point-of-care US in hemophilic care
Several different protocols have been developed for POCUS examination of joints in hemophilic care. One widely used protocol is HEAD-US (Haemophilia Early Arthropathy Detection with Ultrasound), which assesses damage to the knees, elbows, and ankles and can be easily incorporated into the examination routine. It can also detect acute bleeding without the presence of a radiologist. For optimal results, combining the HEAD-US protocol with the HJHS score is recommended. Other protocols include US-US (Universal Simplified Ultrasound) and JADE (Joint Tissue Activity and Damage Exam).
Several studies have examined the yield of US examinations, finding that US conclusions were comparable to MRI conclusions, except for the detection of bone cysts and cartilage loss, where US showed lower sensitivity.
Conclusion
Several methods can be used for a comprehensive evaluation of joint health in hemophilic patients. Point-of-care US examination seems appropriate for inclusion in outpatient practice. POCUS, as an effective and relatively inexpensive method, can be used to assess disease progression, early detection of osteochondral damage and synovial proliferation, detection of acute bleeding, and evaluation of therapy response.
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Source: Di Minno M. N. D., Martinoli C., Pasta G. et al. How to assess, detect, and manage joint involvement in the era of transformational therapies: role of point-of-care ultrasound. Haemophilia 2023; 29 (1): 1−10, doi: 10.1111/hae.14657.
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