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Diagnosis: Collapsing Glomerulopathy

Collapsing glomerulopathy (CG) can be a primary disease (no associated to other alterations or factors), or it can be associated to HIV infection, parvovirus B19, pamidronate, autoimmune diseases, other drug exposure, other infections, malignancies, genetic disorders, atheroembolism, acute vaso-occlusive disorders, and other rare associations. CG is not only a glomerular or podocyte disease, but all renal epithelial cells may be affected. The degree of tubulointerstitial damage varies from case to case and appears more pronounced in those forms where intrinsic epithelial cell damage, such as viral infection, is the related factor. Attempts to identify pathogenic mechanisms in CG have raised a number of hypotheses; however, the mechanisms through which these disorders lead to diffuse podocyte injury are poorly understood. Some investigators have focused their attention on the mitochondria. Mutations in genes encoding proteins involved in the mitochondrial respiratory chain may lead to CG in both mice and humans. Given the crucial role of oxygen availability in mitochondria metabolism, these data suggest that podocytes may be highly sensitive to hypoxic conditions. Recent evidence that hypoxia-inducible factor upregulation in podocytes leads to their proliferation and dedifferentiation in HIV associated nephropathy is striking. Injured podocytes show loss of some podocyte markers, induction of expression of some molecules and transdifferentiation to macrophage lineage.

The term “collapsing glomerulopathy” was used for the first time in 1986, and it was assumed to be a variant of FSGS. However, CG is frequently an aggressive disease, with massive proteinuria and rapidly progressive renal disease. The notoriously more aggressive outcome in CG that in the other variants of FSGS, and the phenotypic features of podocytes suggesting a different pathogenesis, have led some authors to propose that CG is not a variant of FSGS.

See the chapter Focal and Segmental Glomerulosclerosis of our tutorial.

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References

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• Nelson PJ, Bruggeman LA. Collapsing glomerulopathy: beyond serendipity in mouse genetics. Kidney Int. 2009;75(4):353-5. [PubMed link] [Free full text]
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• Barisoni L, Schnaper HW, Kopp JB. A proposed taxonomy for the podocytopathies: a reassessment of the primary nephrotic diseases. Clin J Am Soc Nephrol. 2007;2(3):529-42. [PubMed link][Free full text]
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• Swaminathan S, Lager DJ, Qian X, Stegall MD, Larson TS, Griffin MD. Collapsing and non-collapsing focal segmental glomerulosclerosis in kidney transplants. Nephrol Dial Transplant. 2006;21(9):2607-14. [PubMed link] [Free full text]
• Albaqumi M, Soos TJ, Barisoni L, Nelson PJ. Collapsing glomerulopathy. J Am Soc Nephrol. 2006;17(10):2854-63 [PubMed link] [Free full text]

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