伊库利珠单抗
医学
血栓性微血管病
免疫抑制
移植
肾移植
补体系统
非典型溶血尿毒综合征
肾病
内科学
胃肠病学
免疫学
抗体
疾病
内分泌学
糖尿病
作者
Anna Duval,Jérôme Olagne,Augustin Obrecht,Gabriela Gautier-Vargas,Peggy Perrin,Bruno Moulin,Véronique Frémeaux‐Bacchi,Sophie Caillard
标识
DOI:10.1016/j.ajt.2023.05.031
摘要
Crescentic forms of immunoglobulin A nephropathy (IgAN) are rare but can be associated with rapid kidney failure and a high rate of end-stage renal disease despite immunosuppression therapy. Complement activation has emerged as a key driver of glomerular injury in IgAN. Therefore, complement inhibitors may be a rational treatment option in patients unresponsive to first-line immunosuppressive therapy. Here, we describe the case of a 24-year-old woman presenting with crescentic IgAN recurrence a few months after living kidney transplantation. Considering the dramatic graft failure accompanied by malignant hypertension and thrombotic microangiopathy features worsening after a first-line of high-dose steroids and 3 sessions of plasma exchanges, eculizumab was started as a rescue therapy. For the first time, the clinical response to eculizumab was highly successful, with a complete graft recovery without any relapse after 1 year of treatment. Further clinical studies are strongly needed to specify which patients might benefit from terminal complement blockade.
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