The relevance of MRI findings in joints of persons with haemophilia: Insights from the last decade

Abstract

Most bleeding episodes in persons with haemophilia (PwH) occur in the large synovial joints (elbows, knees, and ankles). Recurrent joint bleeding eventually leads to irreversible haemophilic arthropathy, which causes pain, reduced functionality, and thus reduced quality of life. Prophylactic treatment prevents most bleeding episodes. 1 Even in the absence of clinically overt joint bleeding, long-term progression to arthropathy is observed. Subclinical bleeding and inflammation are therefore thought to contribute to the development of arthropathy. 2-4 Detection of these subclinical processes is becoming increasingly important in the prevention of arthropathy in PwH as overt spontaneous joint bleeding is almost completely avoided by prophylaxis with new (non-factor) replacement therapies. 5 Magnetic resonance imaging (MRI) is considered the gold standard for evaluation of early blood-induced joint changes in PwH. In 2005, the International Prophylaxis Study Group (IPSG) published compatible scales for progressive and additive MRI assessment based on the Denver MRI score and the European MRI score. 6 These scores were combined in a comprehensive scoring scheme in 2012. 7 Based on an appraisal of the original IPSG MRI scale, 8 the score is now updated to the IPSG MRI Scale version 2.0 by Lundin and colleagues. 9 To provide more insight on the clinical relevance of MRI findings, we provide an overview of research on the clinical relevance of MRI findings evaluated by IPSG MRI Scale version 2.0.