Immune-mediated entities of (primary) focal segmental glomerulosclerosis

Cell Tissue Res. 2021 Aug;385(2):423-434. doi: 10.1007/s00441-021-03454-3. Epub 2021 Apr 27.


Focal segmental glomerulosclerosis (FSGS) represents a glomerular scar formation downstream of various different mechanisms leading to podocytopathy and podocyte loss. Recently, significant advances were made in understanding genetic factors, podocyte intrinsic mechanisms, and adaptive mechanisms causing FSGS. However, while most cases of nephrotic FSGS are being treated with immunosuppressants, the underlying immune dysregulation, involved immune cells, and soluble factors are only incompletely understood. Thus, we here summarize the current knowledge of proposed immune effector cells, secreted soluble factors, and podocyte response in immune-mediated (primary) FSGS.

Keywords: Immune cell; Immune epithelial interaction; Podocyte; Primary focal segmental glomerulosclerosis; Soluble factor.

Publication types

  • Review

MeSH terms

  • Animals
  • Glomerulosclerosis, Focal Segmental / immunology*
  • Glomerulosclerosis, Focal Segmental / pathology
  • Humans