Ronco Pierre, Debiec Hanna
Unité INSERM UMR S 702, UPMC Université Paris 6, Assistance Publique-Hôpitaux de Paris, Hôpital Tenon, Paris.
Bull Acad Natl Med. 2012 Nov;196(8):1613-22; discussion 1622-3.
Maternal-fetal alloimmunisation with antenatal glomerulopathies (FMAIG) is a recently described allo-immune disorder that results from the production of maternal antibodies which cross the placenta, bind to fetal glomerular podocytes and thereby cause renal dysfunction. The pathogenic antibodies are directed against CD10/neutral endopeptidase (NEP). The infant's mother is apparently healthy but is genetically NEP-deficient, and thus becomes immunized against CD10/NEP expressed by placental cells during her first pregnancy. This disease, that we have now diagnosed in five families, is the first described organ-specific disorder due to maternal-fetal allo-immunisation. Because future pregnancies in CD10/NEP-immunized mothers are at high risk for the fetus, antigen-driven therapies aimed at eliminating pathogenic antibodies are urgently needed. This will require identification of the pathogenic epitopes born by the antigen.
伴有产前肾小球病的母胎同种免疫(FMAIG)是一种最近描述的同种免疫性疾病,它是由母体产生的抗体引起的,这些抗体穿过胎盘,与胎儿肾小球足细胞结合,从而导致肾功能障碍。致病抗体针对CD10/中性内肽酶(NEP)。婴儿的母亲表面上健康,但在基因上缺乏NEP,因此在她第一次怀孕时,针对胎盘细胞表达的CD10/NEP产生了免疫。我们现在已在五个家庭中诊断出这种疾病,它是首次描述的由母胎同种免疫引起的器官特异性疾病。由于CD10/NEP免疫的母亲未来怀孕时胎儿面临高风险,因此迫切需要旨在消除致病抗体的抗原驱动疗法。这将需要鉴定抗原所携带的致病表位。