ORIGINAL ARTICLE Paediatrics Assessment of the progression of haemophilic arthropathy in children H. PERGANTOU,* H. PLATOKOUKI,* G. MATSINOS,  O. PAPAKONSTANTINOU, à A. PAPADOPOULOS,§ P. XAFAKI,* D. PETRATOS  and S. ARONIS* *Haemophilia Centre – Haemostasis Unit, Aghia Sophia Children’s Hospital;  2nd Department of Orthopaedics, Aghia Sophia Children’s Hospital; à2nd Department of Radiology, Attikon Hospital, University of Athens; and §Department of Radiology – ‘‘Iatropolis Magnetic Resonance Imaging’’ Diagnostic Centre, Athens, Greece Summary. Arthropathy is considered as an irrevers- ible and progressive complication in patients with haemophilia, even in children on prophylaxis. To estimate the progression of haemophilic arthropathy, 85 joints of 24 boys with severe (n = 18) and moderate (n = 6) haemophilia (A: 22, B: 2) were investigated with clinical examination, X-rays and magnetic resonance imaging (MRI) at two time periods (time 0 and 1). Patients’ age at time 0 was 10.5 ± 3.6 years and time elapsed to time 1 was 3.8 ± 1.4 years. At time 0: all investigated joints had more than three bleeds. Sixteen boys were on secondary prophylaxis for 5.4 ± 2.8 years. Clinical score (a modification of World Federation of Hae- mophilia’s scale): 2.0 ± 3.6, X-ray score (Pettersson): 2.1 ± 2.8, MRI score (Denver): 4.5 ± 3.8. After the first evaluation, prophylaxis was intensified in 11 children and initiated in four. At time 1: clinical score: 1.5 ± 3.1, X-ray: 1.7 ± 2.7, MRI score: 5.1 ± 4.1. On average, the clinical and X-ray scores showed a significant improvement (26% and 40% of the joints respectively, P < 0.01) and the number of haemarthroses evidenced a threefold reduction from time 0 to 1 (P < 0.01), findings that could be associated with the modification of prophylaxis after time 0. MRI findings showed deterioration in 34% of the joints. Conversely, 14 joints (16.5%) with mild or moderate synovitis without cartilage degradation at time 0 showed an improvement at time 1. The information carried by the three scales could be divided into information shared by the three scores and information specific to each score, thus giving a more complete picture of joint damage caused by bleedings. Keywords: clinical score, haemophilia, haemophilic arthropathy, magnetic resonance imaging score, prophylaxis, X-ray score Introduction Joint bleedings are the most characteristic symptoms of haemophilia occurring with an annual frequency of 20–40 episodes in patients with the severe type of the disease receiving on demand treatment. Haem- arthroses usually appear when the child is learning to walk, but the inter-individual age of the first joint bleed varies and has been estimated between 1 and 5 years, with a higher frequency between 1.2 and 2 years of age [1]. In severe haemophilia, 92% of all bleeding episodes occur in joints and 80% of them in ankles, knees and elbows. Isolated haemarthroses are usually and relatively harmless because once the blood has been reabsorbed and the swelling has diminished, normal joint mobility and function is restored and no radiographical changes are detected. Recurrent joint haemorrhages cause damage to various joint elements with the eventual development of arthropathy. Haemophilic arthropathy is the most important cause of morbidity in patients with severe haemophilia. It is characterized by synovial hypertrophy, haemosiderin depositions, cartilage Correspondence: Helen Pergantou, Haemophilia Centre-Haemo- stasis Unit, Aghia Sophia Children’s Hospital, Thivon/M.Assias, Goudi, 115 27 Athens, Greece. Tel.: +30 210 746 7769; fax: +30 210 779 6405; e-mail: haemofilia@paidon-agiasofia.gr, helenpergantou@in.gr Accepted after revision 18 August 2009 Haemophilia (2010), 16, 124–129 DOI: 10.1111/j.1365-2516.2009.02109.x 124 Ó 2009 Blackwell Publishing Ltd