Rheumatological management of patients with hemophilia. Part 1: joint manifestations.

The advent of factor VIII and IX replacement therapy has radically changed the physiognomy of hemophilia. In patients with no inhibitors, early replacement therapy shortens the immobilization and decreases the structural and functional alterations related to recurrent hemarthrosis. Routine prophylactic replacement therapy before or after the first episode of hemarthrosis is still rarely used in France. Recurrent hemarthrosis in the same joint can cause synovitis and chronic arthropathy. Injection synovectomy is now the preferred treatment, as opposed to secondary prophylactic replacement therapy and to arthroscopic or open synovectomy. The palliative treatment of chronic arthropathy is difficult and rests on analgesics and rehabilitation therapy, with orthotic devices and/or surgery where appropriate. The treatment of hemophilia is far more difficult in patients with inhibitors and, consequently, considerable hope is being placed in gene therapy, whose first results are encouraging.