AB1133 Role of the rheumatologist in a regional reference haemophilia unit

Background Haemophilia is an X-linked hereditary bleeding disorder caused by deficiency in coagulation factor VIII (FVIII), in haemophilia A (HA), and factor IX in haemophilia B (HB). They are classified as severe, moderate or mild, depending on the level of coagulation factor deficiency. Men are affected clinically by the disease, and women, who are carriers, usually remain asymptomatic. HA is more common than HB (from 80% to 85% of all cases). Their bleeding complications primarily affect the musculoskeletal system. Hemarthrosis is the major hemophilia-related complication, responsible for a particularly debilitating chronic arthropathy, in the long term, affecting mainly the load joints (knees, ankles and elbows). In addition to clotting factor concentrates, usually prescribed by the haematologist, The management of acute hemarthrosis and chronic arthropathy requires a close collaboration with rheumatologists. This collaboration is the key to effectively preventing hemarthrosis, managing acute joint bleeding episodes, assessing joint function, and actively treating chronic arthropathy. Objectives To analyse the clinical characteristics, extent of joint involvement and associated comorbidity of a cohort of patients with hemophilic arthropathy. Methods This is a retrospective study, carried out in the Haemophilia Unit of our hospital (regional reference), in patients with moderate to severe haemophilia A and B, with hemophilic arthropathy, seen in consultation with episodes of joint bleeding (2007–2017). Severity of haemophilia was defined based on determined by the percentage of FC activity (VIII and IX), moderate from 1% to 5%, severe Results We included 88 patients (87 men and 1 symptomatic carrier woman with decreased levels of factor VIII), mean age 31±17 years. HA (severe 56%, moderate 26%), HB (severe 14%, moderate 1%). The target joint: knee 51%, followed by ankle 26%, elbow 13% and other 7% (5 shoulders and 2 wrists). In 61 patients, magnetic resonance imaging (MRI) was performed: synovial hypertrophy 9%, hemosiderin deposits (in acute stage of joint bleeding) 2% and structural alteration (erosions and subchondral cysts, loss of focal cartilage) 68%. A radioisotope synoviorthesis was made to 18 patients: 12 with sulfide 186 Re colloidal (5 ankles, 4 knees, 2 elbows) and 6 with 90 Y colidal citrate (4 knees, 3 ankles), having a decrease of 74% (range 59%–100%) in the number of hemarthrosis in the 3 subsequent months. Total knee replacement was needed in 13% of the patients (7 with HCV liver disease and in 6 HCV liver disease and coexistence with HIV). They have infection due to HCV 33%, HIV 25% and HBV 6%. Conclusions This study highlights the extent of joint damage in haemophiliac patients as well as the high comorbidity of HCV and HIV infections. The experience of a monographic Haemophilia consultation, with the participation of different specialties (being fundamental the rheumatologist), benefits the multidisciplinary approach of these patients, being the results obtained in our series concordant with the described in the literature. Disclosure of Interest None declared