Immune podocyte injury in autoimmune glomerular diseases

自身免疫性肾小球疾病中的足细胞免疫损伤

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Abstract

Autoimmune glomerular diseases (AGDs) are immune dysregulation-driven disorders of the glomerulus and a major cause of chronic kidney disease (CKD) and end-stage kidney disease (ESKD). The glomerular filtration barrier, formed by fenestrated endothelial cells, podocytes, and the glomerular basement membrane (GBM), is indispensable for renal homeostasis. Podocytes are terminally differentiated epithelial cells and are difficult to replace once injured. In susceptible individuals, maladaptive activation of humoral and cellular immunity promotes autoantibody formation, immune complex deposition, and complement activation within the glomerulus, leading to early proteinuria and progressive loss of kidney function. Across clinically heterogeneous AGDs, podocytes represent a key convergence point at which immune effector signals are translated into structural and functional barrier failure. Accumulating evidence further suggests that podocytes are not merely passive targets but active "immune podocytes" capable of engaging innate danger-sensing pathways and adopting adaptive immune-like programs that shape local inflammation and disease evolution. This Review synthesizes current advances in immune-mediated podocyte injury, with emphasis on complement-linked signaling, podocyte-intrinsic inflammatory circuits, and podocyte-associated immune interactions. We relate these mechanisms to cytoskeletal remodeling, organelle stress, and regulated cell-death pathways that culminate in podocyte depletion and glomerulosclerosis. We also discuss podocyte protective responses and emerging opportunities for precision, podocyte-centered therapeutics to improve long-term outcomes in AGDs.

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