Current Views on Collapsing Glomerulopathy

Collapsing Glomerulopathy in 19 Patients with Systemic Lupus Erythematosus or Lupus-Like Disease

“…By immunohistochemistry, the glomerular epithelial cells show a proliferative (100% of cases with +Ki-67 labeling) and dedifferentiated phenotype, displaying loss of synaptopodin and podocin localized mainly in the areas of CG in 83% and 80% of cases tested, respectively, whereas WT-1 preservation is seen in 64% of the cases. The pattern of staining for synaptopodin and podocin is consistent with other nonlupus-related cases of idiopathic CG or HIVAN (20), but WT-1 preservation seems to be unique to an autoimmune etiology, because idiopathic cases of CG typically also show a loss of WT-1 (20). WT-1 positivity in CG cases with SLE was also noted in one other reported case (18).…”

“…Previous reports indicate widespread loss of synaptopodin and WT-1 markers in viral and idiopathic related CG with less dysregulation and dedifferentiation in reactive and genetic cases (20). In this study, WT-1 staining was preserved in 9 of 14 cases (64%), with loss of synaptopodin and podocin in the collapsed segments in 10 of 12 (83%) cases and 8 of 10 (80%) cases, respectively (Figure 4, A-C and Table 2).…”

“…Immunohistochemical stains for podocyte proteins have been documented in idiopathic CG and HIV-associated nephropathy (HIVAN) but not CG associated with autoimmune disease (20). Previous reports indicate widespread loss of synaptopodin and WT-1 markers in viral and idiopathic related CG with less dysregulation and dedifferentiation in reactive and genetic cases (20).…”

Glomerular Epithelial Stem Cells

“…72 That is, crescentic glomerulonephritis presents with podocyte damage in an inflammatory environment characterized by nephritic features, whereas collapsing glomerulopathy often presents with nephrotic features. 62,63,72,73 Although epithelial cell proliferation is most characteristic and prominent in crescentic glomerulonephritis or collapsing glomerulopathy, some epithelial cell proliferation is also observed in histopathologic lesions typically found in other podocytopathies, such as the tiplesion. 62 Interestingly, we recently demonstrated that in the tip lesion, as well as in those FSGS which are characterized by mild levels of hyperplasia, glomerular epithelial stem cells are the main constituents of the proliferative lesion.…”

Adult Stem Cells in Tissue Homeostasis and Disease

“…In the glomerulus, the response to podocyte injury may cause aberrant epithelial cell proliferation, hypercellular lesions formation and Bowman's space obliteration, as seen in collapsing glomerulopathy and in crescentic glomerulonephritis (Albaqumi & Barisoni, 2008;Thorner, et al, 2008). Until now, theories explaining the origin of aberrant epithelial cells in collapsing glomerulopathy and crescentic glomerulonephritis have been controversial.…”