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Diagnosis: Colapsing Glomerulopathy

Glomeruli present collapsing lesions with severe hypertrophy and hyperplasia of podocytes, the involvement is not diffuse.

Another diagnosis possibility is diffuse mesangial sclerosis, but it is characterized by early onset nephrothic syndrome, generally before 2 years of age. Very important here is the fact that lesions are collapsing and not sclerosing.

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. 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.

Visit the chapter: Focal and Segmental Glomerulosclerosis of our Tutorial.

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References

• Kanodia KV, Vanikar AV, Patel RD, Suthar KS, Nigam LK, Patel HV, Kute V, Trivedi HL. Collapsing Glomerulopathy: A Single Centre Clinicopathologic Study of Seven Years. J Clin Diagn Res. 2016;10(4):EC15-7. [PubMed link]
• Ahuja A, Gupta R, Sharma A, Bagga A, Bhowmik DM, Agarwal SK, Dinda AK. Idiopathic collapsing glomerulopathy: A clinicopathologic analysis of 30 cases. Indian J Nephrol. 2014;24(4):239-42. [PubMed link]
• Chugh SS, Clement LC. Telomerase at the center of collapsing glomerulopathy. Nat Med. 2012;18(1):26-7 [PubMed link]
• Howie AJ. Problems with 'focal segmental glomerulosclerosis'. Pediatr Nephrol. 2011;26(8):1197-205. [PubMed link]
• Canaud G, Bruneval P, Noël LH, Correas JM, Audard V, Zafrani L, Rabant M, Timsit MO, Martinez F, Anglicheau D, Thervet E, Patey N, Legendre C, Zuber J. Glomerular collapse associated with subtotal renal infarction in kidney transplant recipients with multiple renal arteries.Am J Kidney Dis. 2010 Mar;55(3):558-65. [PubMed link]
• Nelson PJ, Bruggeman LA. Collapsing glomerulopathy: beyond serendipity in mouse genetics. Kidney Int. 2009;75(4):353-5. [PubMed link] [Free full text]
• Gulati A, Sharma A, Hari P, Dinda AK, Bagga A. Idiopathic collapsing glomerulopathy in children. Clin Exp Nephrol. 2008;12(5):348-53. [PubMed link]

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