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논문분류	춘계학술대회 초록집
제목	Abnormal renal presentation of a common autoimmunity disease
저자	Dina Sallam
출판정보	2024; 2024(1):
키워드
초록	Background and Aims: The long-term complications of COVID-19 infection & Multisystem inflammatory syndrome in children (MIS-C) on immune system are still unknown, however there’re reports on development of autoimmune disease, highlighting their immune-related nature. MIS-C can induce Thrombotic Microangiopathy (TMA), by inducing endothelial dysfunction, platelet activation, & microthrombi formation, whereas SLE induces TMA/Thrombotic thrombocytopenic purpura (TTP), with ADAMTS13 activity < 10%, or 2ry TMA with ADAMTS13 activity ≥ 10%, where SLE/TTP carries a bad prognosis. Our case is one of its kind, where a 12-year- old girl, who was previously infected with SARS-CoV-2, presented with new-onset SLE, associated MIS-C & 2ry TMA/TTP. COVID serology was +ve for IgG, & -ve for IgM, & -ve SARS-CoV-2 PCR, a diagnosis of MIS-C was postulated. Further investigations revealed low C3, C4, +ve ANA, anti-ds DNA & anti-phospholipid Antibodies. Brain imaging showed left cerebellar calcification. Echocardiography showed biventricular dysfunction with EF 30%, where the diagnosis of SLE according to Euler/ACR criteria was confirmed, with 2ry TMA. Emergency hemodialysis, PRBCs transfusion, pulse steroid with total plasma exchange (TPE), were initiated without marked improvement. Rituximab was started on 4th day, but she had generalized convulsion, arrythmia & sudden death occurred. Results of ADAMTS13 activity was < 1%, with +ve autoantibodies, so diagnosis of TMA/TTP was confirmed. Conclusion: Herein we raise the question of cause of TMA/TTP, was it 2ry only SLE, or to the previous COVID-19 infection & MIS-C, and if this SLE itself was 2ry also to the previous COVID infection, or all it was by chance?
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