MRI assessment of haemophilic arthropathy

Abstract: Haemophilia is a recessive genetic bleeding disorder in which coagulation time is prolonged due to the absence or malfunction of either of the serum proteins Factor VIII or IX. Joint destruction following repeated haemarthroses is the main cause of morbidity in haemophiliacs, and such arthropathy can be prevented or postponed by implementing prophylactic therapy with factor concentrate at an early age. Prophylaxis is the recommended treatment for severe haemophilia, but there is no consensus as to the optimal or the most cost effective prophylactic regimen. New sensitive methods are needed to monitor and evaluate haemophilia care. Magnetic resonance imaging (MRI) is a modality with superior capacity to visualize joint pathology. We designed a new additive MRI scoring method for haemophilic arthropathy (the European MRI score) that can monitor reversible and irreversible components of the joint disease separately. We compared this approach with a previously presented progressive method (the Denver MRI score), and then analysed the correlation between the results obtained with those two methods and clinical data on joint bleeds and physical joint examinations. Based on the findings, we developed a combined scoring scheme (the compatible MRI scales). Moreover, we investigated the intra- and interobserver agreement of the MRI scores, and the value of gadolinium contrast agent for MRI scoring of haemophilic arthropathy. The European MRI scoring method is useful for sensitive assessments of the progression of haemophilic arthropathy, and it is more efficient than the Denver method in differentiating the arthropatic changes. Nonetheless, the European and Denver scores are highly correlated with each other, and they show the same degree of correlation with data on number of joint bleeds and physical joint examination. The compatible MRI scales can be applied to achieve practical assessment of early arthropathy and for assessment of the progression of the disease over time. Reliable MRI scoring is feasible, provided uniform imaging protocols are utilized and the evaluation is conducted by specially trained radiologists. Gadolinium contrast agent is not routinely indicated for MRI scoring of haemophilic arthropathy