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Haemophilic Arthropathy — SCE Rheumatology MCQ

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ModerateOsteoarthritisHaemophilic ArthropathySCE Rheumatology

A 40-year-old man with haemophilia A has experienced recurrent haemarthroses of the left knee. Radiographs show joint-space loss, subchondral cysts and epiphyseal overgrowth. Which pathological process best explains the development of his arthropathy?

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Correct answer: BRecurrent haemarthroses cause iron-mediated chondrocyte injury and proliferative synovitis, with neoangiogenesis and progressive cartilage and bone destruction

The correct answer is B. Repeated intra-articular bleeding exposes cartilage and synovium to blood-derived iron. Iron promotes oxidative chondrocyte injury and accumulates as haemosiderin within the synovium, driving synovial hypertrophy, inflammatory mediator release and neoangiogenesis. The abnormal vascular synovium is prone to further bleeding, creating a cycle of haemarthrosis, synovitis and progressive cartilage and subchondral bone destruction. Epiphyseal overgrowth reflects chronic hyperaemia, particularly when bleeding began before skeletal maturity. Crystal deposition may cause a separate arthropathy but is not the characteristic mechanism here. Infection and autoimmune synovitis are alternative causes of destructive monoarthritis, not consequences of factor VIII deficiency. Osteonecrosis can complicate haemophilia but does not explain the characteristic blood-induced synovial and cartilage pathology.

Reference: Melchiorre D, Manetti M, Matucci-Cerinic M. Pathophysiological Role of Synovitis in Hemophilic Arthropathy Development: A Two-Hit Hypothesis. Frontiers in Physiology. 2020. https://pubmed.ncbi.nlm.nih.gov/32581836/