I Monteagudo Sáez

AB0721 Transfer of systemic sclerosis after allogeneic bone marrow transplantation

Background It is accepted that donor-derived immunity is transferred with allogeneic bone marrow transplantation (BMT)1. Objectives To show evidence of the transfer of systemic sclerosis by allogeneic BMT. Methods In this report we describe a pacient with T acute lymphoblastic leukaemia who underwent BMT and developed systemic sclerosis. Results 34-year-old man in complete remission from a T acute lymphoblastic leukaemia treated with allogeneic BMT from his mother in february of 2012. First seen in november 2017 for digital ulcers that appeared one year before. He presented two necrotic ulcers: one on the second finger of the left hand and other on the third finger of the right hand (IMAGE 1). He was admited to receive intravenous prostaglandins and complete the study. After the BMT he developed Raynaud’s phenomenon and in the examination he only presented facial and corporal telangiectasia, attributed before to chronic graft versus host disease (cGVHD). The analysis showed ANA 1/640 centromere pattern, anticentromere antibodies, reumatoid factor (RF) (652 UI/mL) and C3 of 86.4 mg/dL. Previously to the BMT he had negative ANA, but we do not know the rest of the previous autoimmunity. On the videocapillaroscopy we observe an active scleroderma pattern (IMAGE 2–3). He was diagnosed with systemic sclerosis based on Raynaud’s phenomenon, digital ulcers, anticentromere antibodies and abnormal nailfold capillaries. He had not familiar background of connective tissue diseases, but his mother presented Raynaud’s phenomenon since she was thirty. So we studied her. She presented facial telangiectasia, puffy fingers and fingertip pitting scars and the same autoantibodies: ANA 1/320 centromere pattern, anticentromere antibodies, RF (159 UI/mL) and consumption of C3 (74.3 mg/dL) and C4 (7.6). We do not have previous autoimmune studies of her. On the videocapillaroscopy we observed a late scleroderma pattern (IMAGE 4–5). She was also diagnosed with systemic sclerosis, based on Raynaud’s phemomenon, puffy fingers, fingertip pitting scars and facial telangiectasia, anticentromere antibodies and abnormal naifold capillaries. Conclusions Experimental animal studies and human clinical reports have described the transfer of immune-mediated diseases from affected donors to unaffected recipients, because of that the importance of screening this diseases in the donor before a BMT2. To our knowledge, this will be the first described case of transmission of systemic sclerosis by this mechanism. However, other explanations should also be taken into consideration. This include recipient’s own persistent intrathymic lymphocyte population that may produce autoantibodies and autoimmunity in the context of cGVHD. However, this last hypothesis is not supported since there is a familiar background and presence of anticentromere antibodies. References [1] Sherer, Y. and Shoenfeld, Y. (1998). Autoimmune diseases and autoimmunity post-bone marrow transplantation. Bone Marrow Transplantation. [2] Hough, R.(2005). Haemopoietic stem cell transplantation in autoimmune diseases: a European perspective. British Journal of Haematology. Disclosure of Interest None declared

AB0668 Treatment of thrombotic events in behÇet disease: a systematic literature review

Background Behçet’s disease (BD) is a systemic disease which etiopathogenesis is largely unknown. It is characterised by a wide variety of clinical manifestations. Venous disorder is a serious manifestation being potentially life-threatening. There is little evidence on the management of the venous complications in BD. Objectives To perform a systematic literature review on the treatment used in venous thrombotic events in BD. Methods The objective was reformulated according to the PICO approach. Several synonyms for the main components (i.e. Behçet, thrombosis, treatment) were used. Search limits were applied for humans. The literature search was performed in Medline and Embase from databases inception to 1st November 2017. Only articles in English and Latin languages were retained. We excluded abstracts, reviews and letters. From the selected studies, data about the venous involvement and treatments were retired using a predefined data collection form.Abstract AB0668 – Table 1 Treatment N° articles (%) Anticoagulant 22 (78.6) Antiplatelet 9 (33.3) Corticosteroids 22 (78.6) Immunosuppressive 25 (89.3) Cyclophosphamide 16 (57.1) Azathioprine 16 (57.1) Cyclosporine A 5 (17.9) Mycophenolate mofetil 2 (7.1) Interferon alpha 2 (7.1) Methorexate 4 (14.3) Hydroxychloroquine 1 (3.6) Anti-TNF alpha 4 (14.3) Colchicine 11 (39.3) Thalidomide 1 (3.6) Dapsona 1 (3.6) Fibrinolytic 3 (10.7) Surgery 7 ((25.9) Results The literature search resulted in 1552 articles, of which 632 were captured in Medline and 920 in Embase. Figure 1 shows the study flow-chart for article selection. The main reasons for article exclusion after full-text review were the lack venous involvement and the lack of explanation of venous involvement treatment. 28 articles reporting 1904 patients were included in qualitative analysis. The mean (range; SD) duration time between the disease onset and the vascular onset was evaluated in 15 articles and was 4.9 (1.2–9.3; 2.7) years. Superficial thrombosis was evaluated in 6 (21.4%) articles, profound thrombosis in 19 (67.9%) articles, cerebral in 7 (25%), inferior or superior cava vein in 15 (53.6%) and Budd-Chiari syndrome in 8 (28.6%) articles. Table 1 shows the treatments described in the selected articles. Treatment response was evaluated in 20 (71.4%) articles; in 7 of these treatments response was evaluated in a subjective way. In total, 52 (2.7%) deaths were reported in relation to BD. In 319 (16.7%) patients, partial efficacy or recurrence of thrombosis was reported. Considering the heterogeneity of the reported data and the variability in the measures of treatment response, predictors of mortality risk cannot be analysed. However, in the reviewed articles, a higher mortality rate was observed in in patients with hepatic involvement due to Budd-Chiari syndrome. We have also observed a higher risk for the development of venous thrombosis in patients with patergia phenomenon and male sex. Two studies suggested that immunosuppressive treatment concomitant with anticoagulant treatment is associated with a lower risk of thrombosis relapse compared with anticoagulant treatment alone.Abstract AB0668 – Figure 1 Conclusions There is a great variability in the treatment of venous thrombosis related to Behçet’s disease. Budd-Chiari syndrome seems to be related to a worse prognosis of the disease. Disclosure of Interest None declared

Mujer de 65 años con artritis reumatoide de larga evolución, síndrome constitucional, fiebre y púrpura cutánea

Caso clinico PMB es una mujer de 65 anos de edad diagnosticada de artritis reumatoide de 16 anos de evolucion, erosiva, nodular y seropositiva. El curso de su enfermedad fue progresivo, a pesar de los tratamientos utilizados. En la revision, 4 meses antes de su ingreso, no habia sinovitis y presentaba un hemograma normal, VSG 21 mm, proteina C reactiva (PCR) 0 y factor reumatoide 887 UI/ml. En el ultimo mes refiere astenia, perdida de 5 kilos de peso y purpura en las piernas. En las ultimas 2 semanas han aparecido ulceras cutaneas en los tobillos y parestesias en ambos pies, por lo que ha acudido a su consulta de atencion primaria. Ingresa por presentar fiebre de 24 horas de evolucion, disnea de moderados esfuerzos y dolor retroesternal que aumenta con la inspiracion y el decubito. La paciente esta consciente, orientada y bien hidratada. La temperatura es de 38,5 °C, la tension arterial 156/84 y el pulso ritmico a 96 lpm. El examen de la cabeza y el cuello es normal. No presenta taquipnea en reposo y la auscultacion pulmonar es normal. La auscultacion cardiaca revela un roce leve. El examen abdominal es normal. Presenta nodulos reumatoides en ambos codos y una purpura palpable en ambas piernas, con 2 ulceras maleolares en el tobillo derecho y una en el izquierdo, ovaladas y de 2x4 cm. Los dedos de los pies estan azulados y frios, uno de ellos con necrosis distal, pero los pulsos son normales y no hay edema. Algunos dedos presentan infartos periungueales. El examen neurologico revela disestesia e hipoestesia en ambos pies. La paciente no presenta sinovitis y solo la flexion forzada de ambas rodillas desencadena dolor, aunque hay desviacion cubital en metacarpofalangicas, pulgares en Z, anquilosis de ambas munecas y pies planos triangulares. En el estudio realizado ambulatoriamente presenta VSG 96 mm, PCR 5 mg/dl, anemia normocitica y normocromica, bioquimica y orina normales, hipocomplementemia, anticuerpos antinucleares (ANA) 1:80 con patron homogeneo y factor reumatoide 2568 UI/ml.