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Nat Rev Nephrol. 2016 Dec;12(12):768-776. doi: 10.1038/nrneph.2016.147. Epub 2016 Oct 17.

Minimal change disease and idiopathic FSGS: manifestations of the same disease.

Author information

1
Department of Nephrology, Radboud University Medical Center, Nijmegen, Netherlands.
2
Department of Pathology, Radboud University Medical Center, PO Box 9101, 6500 HB, Nijmegen, Netherlands.
3
Department of Nephrology and Clinical Immunology, University Hospital of the RWTH Aachen University, PauwelsstraƟe 30, 52074 Aachen, Germany.

Abstract

Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the key histological findings in patients with idiopathic nephrotic syndrome (INS). Although MCD and idiopathic FSGS are often considered to represent separate entities based on differences in their presenting characteristics, histology and outcomes, little evidence exists for this separation. We propose that MCD and idiopathic FSGS are different manifestations of the same progressive disease. The gradual development of FSGS in patients with non-remitting or relapsing INS has been well documented. Moreover, FSGS is the uniform result of substantial podocyte loss in animal models, and a common feature of virtually all progressive human glomerulopathies. As evidence suggests a common aetiology, the pathogenesis of MCD and idiopathic FSGS should be studied together. In clinical trials, idiopathic FSGS should be considered to represent an advanced stage of disease progression that is less likely to respond to treatment than the earlier stage of disease, which is usually defined as MCD.

PMID:
27748392
DOI:
10.1038/nrneph.2016.147
[Indexed for MEDLINE]

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