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Haemophilic arthropathy from A to Z

Haemophilic arthropathy is still a major cause of morbidity in patients with severe haemophilia A and B. This condition results from repetitive spontaneous haemarthroses that trigger a chronic, progressive and irreversible inflammatory response [1]. Acute haemarthrosis leads to chronic synovitis and ultimately to degenerative arthritis. Bleeding episodes are prevented or treated by intravenous replacement of the deficient clotting factor [2]. However, the development of inhibitors against the administered clotting factor in some haemophilia patients may render replacement therapy ineffective. Thus, severe haemophilia patients presenting with inhibitors are in the highest risk category for developing arthropathy, and their management represents a significant challenge, particularly when orthopaedic surgery is required. The development of agents capable of bypassing factor VIII and factor IX in the coagulation cascade such as recombinant activated factor VII (rFVIIa, NovoSeven ; NovoNordisk A/S, Bagsvaerd, Denmark) or activated prothrombin complex concentrate (FEIBA ; Baxter, Vienna, Austria) has dramatically improved the treatment and prevention of repetitive bleedings in these patients [3]. In the first randomized clinical trial reported to date on prophylaxis in haemophilia patients with inhibitors, rFVIIa reduced bleeding frequency, hospital stay and absence from work/school, and also improved quality of life compared with conventional on-demand therapy [4]. The management of patients with haemophilic arthropathy can only be envisaged as a multidisciplinary process, involving a group of specialists in physical medicine, physiotherapy, rheumatology, haematology and orthopaedic surgery. This was the idea underlying the scientific symposium held in Lyon, on June 28th 2007 with the unrestricted support from Novo Nordisk A/S. It gathered more than 120 multidisciplinary haemophilia specialists from Europe and North America. The goal was to encourage participants to exchange their views on the optimal management of patients with haemophilic arthropathy, to preserve and improve patient s quality of life. The following four articles summarize the main topics that were discussed: