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

Nature Reviews Nephrology volume 12, pages 768–776 (2016)Cite this article

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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.

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Figure 1: Schematic representation of idiopathic nephrotic syndrome, minimal change disease and idiopathic focal segmental glomerulosclerosis (FSGS).
Figure 2: Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are manifestations of the same disease.

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Acknowledgements

The authors' work is supported by the Dutch Kidney Foundation (OW08, R.J.M and J.F.W.; 14A3D104, B.S.), the Netherlands Organization for Scientific Research (NWO grant 92003587, J.K.D.), the consortium STOP-FSGS by the German Ministry for Science and Education (BMBF 01GM1518A, M.J.M.), and TP17 of the SFB/Transregio 57 “Mechanisms of organ fibrosis” of the German Research Foundation (M.J.M). M.J.M. has also been awarded a Heisenberg Professorship (DFG MO 1082/7-1).

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  1. Marcus J. Moeller and Jack F. Wetzels: M.J.M. and J.F.W. contributed equally to this work.

Authors and Affiliations

  1. Department of Nephrology, Radboud University Medical Center, Nijmegen, Netherlands

    Rutger J. Maas, Jeroen K. Deegens & Jack F. Wetzels

  2. Department of Pathology, Radboud University Medical Center, PO Box 9101, Nijmegen, 6500 HB, Netherlands

    Bart Smeets

  3. Department of Nephrology and Clinical Immunology, University Hospital of the RWTH Aachen University, Pauwelsstraße 30, Aachen, 52074, Germany

    Marcus J. Moeller

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  1. Rutger J. Maas
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Contributions

All authors researched the data for the article, discussed the content and wrote the manuscript. R.J.M. and J.F.W. revised and edited the manuscript before submission.

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Correspondence to Rutger J. Maas.

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Maas, R., Deegens, J., Smeets, B. et al. Minimal change disease and idiopathic FSGS: manifestations of the same disease. Nat Rev Nephrol 12, 768–776 (2016). https://doi.org/10.1038/nrneph.2016.147

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