Definition and Classification of Chronic Kidney Disease According to KDIGO

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. It begins with reactive hypertrophy of the synovium, where early detection allows for preventive strategies that help reduce joint damage and functional limitations, thereby improving the quality of life for patients. Traditional clinical approaches and laboratory tests may not demonstrate the sensitivity and specificity needed for monitoring joint health with current modern hemophilia therapy.

Functional Ability Assessment

Besides physical examination, this includes the HJHS score (Hemophilia Joint Health Score), which captures changes in joint functionality, FISH (Functional Independence Score in Hemophilia), which assesses the patient’s independence, and HAL (Haemophilia Activities List), which identifies problematic activities. It also includes a quality of life questionnaire. Together, these tools provide a basic picture of the patient's joint health, further refined by imaging methods. 

Imaging Methods

The gold standard for imaging hemophilic joints is, of course, magnetic resonance imaging (MRI), which provides a comprehensive view of the joint, including the presence of hemosiderin and subchondral cysts. It can also be used to monitor treatment response. Its disadvantages include cost, use of contrast material, and waiting times, making it difficult to use for preventive imaging of all major joints.

X-ray 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, fast, and non-invasive method of assessing joint changes in hemophilic patients that can be used even by non-radiologists. It can visualize both chronic changes (osteo-chondral damage and synovial hypertrophy) and acute joint issues, such as hemarthrosis. Synovial proliferation can also indicate subclinical bleeding, enabling the examining doctor to detect joint changes even in asymptomatic 'non-bleeding' patients. Hemophilic US protocols do not significantly lag behind MRI in terms of specificity and sensitivity. However, using US requires consideration of the doctor's experience and patient variability.

Point-of-Care US in Hemophilic Care

Several protocols have been developed for POCUS examination of joints in hemophilic care. A very commonly used one is HEAD-US (Haemophilia Early Arthropathy Detection with Ultrasound), which assesses knee and elbow joints and ankles and can easily be incorporated into examination routines. It also allows acute bleeding detection, all without the necessary presence of a radiologist. For optimal results, combining the HEAD-US protocol with the HJHS score is suitable. Other protocols include US-US (Universal Simplified Ultrasound) and JADE (Joint Tissue Activity and Damage Exam).

The yield of US examinations has been investigated in several studies, which show that the conclusions from US were comparable to those from MRI, except for detecting bone cysts and cartilage loss, where US exhibited lower sensitivity.

Conclusion

Several methods can be used for a comprehensive assessment of joint health in hemophilic patients. Incorporating point-of-care US examination into the outpatient practice seems appropriate. As an effective and relatively inexpensive method, POCUS can be used to assess disease progression, early detection of osteo-chondral damage and synovial proliferation, detect acute bleeding, and evaluate 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.