AB0721 Transfer of systemic sclerosis after allogeneic bone marrow transplantation
Authors: A. Silva Riveiro, J.C. Nieto González, J. Martínez Barrio, A. López-Cerón Cofiño, L.A. Torrens Cid, C. Soleto Kharkovskaya, B. Serrano Benavente, R.D. González Benítez, C.M. González Fernández, F.J. López-Longo, I. Monteagudo Sáez
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> It is accepted that donor-derived immunity is transferred with allogeneic bone marrow transplantation (BMT)<sup>1</sup>. <h3>Objectives</h3> To show evidence of the transfer of systemic sclerosis by allogeneic BMT. <h3>Methods</h3> In this report we describe a pacient with T acute lymphoblastic leukaemia who underwent BMT and developed systemic sclerosis. <h3>Results</h3> 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. <h3>Conclusions</h3> 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 BMT<sup>2</sup>. 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. <h3>References</h3> [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. <h3>Disclosure of Interest</h3> None declared