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Identification of cystatin C as a new marker of glomerular filtration rate, and of shrunken pore syndrome – a new kidney disorder defining selective glomerular hypofiltration syndromes – calls for expansion of the international KDIGO guidelines

Åkesson, Anna LU orcid ; Öberg, Carl LU ; Malmgren, Linnea LU orcid ; Nilsson, Christopher LU orcid ; Itoh, Yoshi ; Blirup-Jensen, Søren LU ; Lindström, Veronica LU orcid ; Abrahamson, Magnus LU orcid ; Leion, Felicia LU and Olafsson, Isleifur , et al. (2025) In Scandinavian Journal of Clinical and Laboratory Investigation
Abstract
Cystatin C was identified as a marker of glomerular filtration rate (GFR) in 1979, and the parallel analysis of cystatin C and creatinine led to the identification of shrunken pore syndrome (SPS) ? a new kidney disorder ? in 2015. Since then, it has been shown that cystatin C in many aspects is superior to creatinine as a marker of GFR and cardiovascular risk. SPS, an entity within the selective glomerular hypofiltration syndromes (SGHS), has been demonstrated to be associated with a strong increase in morbidity and mortality in several populations. Despite the seriousness of SPS and SGHS, and the availability of potential treatments, many patients with these conditions remain undiagnosed, due to the limitations of the international Kidney... (More)
Cystatin C was identified as a marker of glomerular filtration rate (GFR) in 1979, and the parallel analysis of cystatin C and creatinine led to the identification of shrunken pore syndrome (SPS) ? a new kidney disorder ? in 2015. Since then, it has been shown that cystatin C in many aspects is superior to creatinine as a marker of GFR and cardiovascular risk. SPS, an entity within the selective glomerular hypofiltration syndromes (SGHS), has been demonstrated to be associated with a strong increase in morbidity and mortality in several populations. Despite the seriousness of SPS and SGHS, and the availability of potential treatments, many patients with these conditions remain undiagnosed, due to the limitations of the international Kidney Disease Improving Global Outcomes Organization (KDIGO) guidelines. Given the significant clinical advantages of cystatin C in diagnosing and treating kidney disorders, there is a need to expand the KDIGO guidelines to include cystatin C measurements alongside creatinine at least in the initial patient evaluation but also in follow-up evaluations. This would improve the early detection and management of patients with kidney diseases, ultimately enhancing patient outcomes. The present discourse summarizes the development of this understanding from the original observations in 1979 and 2015 to the latest findings. (Less)
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organization
publishing date
type
Contribution to journal
publication status
epub
subject
in
Scandinavian Journal of Clinical and Laboratory Investigation
pages
11 pages
publisher
Informa Healthcare
external identifiers
  • pmid:40888652
ISSN
0036-5513
DOI
10.1080/00365513.2025.2546320
language
English
LU publication?
yes
id
f1c62ff6-20b4-42c9-a9f9-4fc40e9e2e0c
date added to LUP
2025-09-02 16:32:48
date last changed
2025-09-13 10:23:44
@article{f1c62ff6-20b4-42c9-a9f9-4fc40e9e2e0c,
  abstract     = {{Cystatin C was identified as a marker of glomerular filtration rate (GFR) in 1979, and the parallel analysis of cystatin C and creatinine led to the identification of shrunken pore syndrome (SPS) ? a new kidney disorder ? in 2015. Since then, it has been shown that cystatin C in many aspects is superior to creatinine as a marker of GFR and cardiovascular risk. SPS, an entity within the selective glomerular hypofiltration syndromes (SGHS), has been demonstrated to be associated with a strong increase in morbidity and mortality in several populations. Despite the seriousness of SPS and SGHS, and the availability of potential treatments, many patients with these conditions remain undiagnosed, due to the limitations of the international Kidney Disease Improving Global Outcomes Organization (KDIGO) guidelines. Given the significant clinical advantages of cystatin C in diagnosing and treating kidney disorders, there is a need to expand the KDIGO guidelines to include cystatin C measurements alongside creatinine at least in the initial patient evaluation but also in follow-up evaluations. This would improve the early detection and management of patients with kidney diseases, ultimately enhancing patient outcomes. The present discourse summarizes the development of this understanding from the original observations in 1979 and 2015 to the latest findings.}},
  author       = {{Åkesson, Anna and Öberg, Carl and Malmgren, Linnea and Nilsson, Christopher and Itoh, Yoshi and Blirup-Jensen, Søren and Lindström, Veronica and Abrahamson, Magnus and Leion, Felicia and Olafsson, Isleifur and Bjursten, Henrik and Grubb, David and Herou, Erik and Dardashti, Alain and Sigurjonsson, Johann and Xhakollari, Liana and Laucyte-Cibulskiene, Agne and Pottel, Hans and Strevens, Helena and Damm, Danielle and Förnvik Jonsson, Magnus and Siódmiak, Joanna and Ärnlöv, Johan and Larsson, Anders and Åkerfeldt, Torbjörn and Kultima, Kim and Ridefelt, Peter and Helmersson-Karlqvist, Johanna and Magnusson, Martin and Hansson, Magnus and Sjöström, Anna and Soveri, Inga and Tenstad, Olav and Mårtensson, Johan and Elinder, Carl-Gustaf and Risch, Lorenz and Risch, Martin and Hansson, Lars-Olof and Price, Christopher P. and Nyman, Ulf and Björk, Jonas and Delanaye, Pierre and Bökenkamp, Arend and Christensson, Anders and Grubb, Anders}},
  issn         = {{0036-5513}},
  language     = {{eng}},
  publisher    = {{Informa Healthcare}},
  series       = {{Scandinavian Journal of Clinical and Laboratory Investigation}},
  title        = {{Identification of cystatin C as a new marker of glomerular filtration rate, and of shrunken pore syndrome – a new kidney disorder defining selective glomerular hypofiltration syndromes – calls for expansion of the international KDIGO guidelines}},
  url          = {{http://dx.doi.org/10.1080/00365513.2025.2546320}},
  doi          = {{10.1080/00365513.2025.2546320}},
  year         = {{2025}},
}