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Severe systemic lupus erythematosus with thrombotic microangiopathy: rescue therapy with pegcetacoplan
Christian Stirnkorb1, Verena Ney2, Bhavna Issar2
1Department of Pediatric Nephrology, Olga-Hospital, Clinic of Stuttgart, Kriegsbergstr. 62, 70174, Stuttgart, Germany. c.stirnkorb@klinikum-stuttgart.de.
Abstract:
This report describes the case of a 12-year-old female patient with severe lupus nephritis and refractory thrombotic microangiopathy, who required dialysis for several months despite receiving intensive immunosuppressive treatment, including terminal complement blockade with eculizumab. Following a switch in therapy from eculizumab to pegcetacoplan, there was a significant reduction in haemolytic activity and steady improvement in kidney function. After more than 8 months of haemodialysis, the patient could be weaned off dialysis. This case study highlights the therapeutic potential of pegcetacoplan in the treatment of thrombotic microangiopathy in systemic lupus erythematosus.
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