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Peer-reviewedCC BY 4.0PLOS One

Paradoxical hepcidin–iron dysregulation in primary sclerosing cholangitis

  • Petr Kordac1,2
  • Milan Hajek1
  • Monika Cahova3
  • Mojmir Hlavaty4
  • Petr Sedivy1
  • Monika Dezortova1
  • Dita Pajuelo1
  • Kamila Balusikova5
  • Jan Brezina4
  • Pavel Drastich4
5 affiliations
  1. 1Department of Diagnostic and Interventional Radiology, Institute for Clinical and Experimental Medicine, Magnetic Resonance unit, Prague, Czech Republic
  2. 2Department of Physiology, Faculty of Science, Charles University, Prague, Czech Republic
  3. 3Centre for Experimental Medicine, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
  4. 4Department of Hepatogastroenterology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic
  5. 5Department of Biochemistry, Cell and Molecular Biology, 3rd Faculty of Medicine, Charles University, Prague, Czech Republic
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Abstract

Background: Primary sclerosing cholangitis (PSC) is a chronic, progressive liver disease characterised by biliary inflammation. Its exact aetiology remains unclear. This study aimed to use a combination of diagnostic methods and blood analysis to better understand the metabolic changes accompanying PSC. Methods: PSC patients (n = 60) and healthy controls (n = 60) were evaluated by magnetic resonance imaging and spectroscopy (hepatic fat and iron content), ultrasonography, standard blood analysis expanded with markers of iron metabolism, and colonoscopy. Results: PSC patients exhibited significant liver fibrosis and elevated liver enzymes. Serum hepcidin was increased (p < 0.01), but PSC patients did not show any markers of anaemia. Serum iron concentration and transferrin saturation were increased compared with healthy controls (p < 0.0001 and p < 0.001, respectively) but did not reach levels seen in hemochromatosis. In contrast, the liver iron content was lower in PSC patients (p < 0.0001). Conclusion: PSC patients demonstrated an atypical pattern of iron metabolism: elevated circulating iron and depleted liver iron stores despite increased hepcidin levels. This paradox suggests a disruption of hepcidin-mediated iron regulation, potentially sustaining a proinflammatory environment. Further investigation is warranted to clarify underlying mechanisms and therapeutic implications. Trial Registration: Clinical Trials I.D.: NCT06026449

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Cite

BibTeXRIS
BibTeX
@article{kordac2026paradoxical,
  title = {{Paradoxical hepcidin–iron dysregulation in primary sclerosing cholangitis}},
  author = {Petr Kordac and Milan Hajek and Monika Cahova and Mojmir Hlavaty and Petr Sedivy and Monika Dezortova and Dita Pajuelo and Kamila Balusikova and Jan Brezina and Pavel Drastich},
  journal = {PLOS One},
  year = {2026},
  month = oct,
  doi = {10.1371/journal.pone.0361385},
  url = {https://doi.org/10.1371/journal.pone.0361385},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Paradoxical hepcidin–iron dysregulation in primary sclerosing cholangitis
AU  - Petr Kordac
AU  - Milan Hajek
AU  - Monika Cahova
AU  - Mojmir Hlavaty
AU  - Petr Sedivy
AU  - Monika Dezortova
AU  - Dita Pajuelo
AU  - Kamila Balusikova
AU  - Jan Brezina
AU  - Pavel Drastich
PY  - 2026
DA  - 2026/10/27
JO  - PLOS One
DO  - 10.1371/journal.pone.0361385
UR  - https://doi.org/10.1371/journal.pone.0361385
AB  - Background: Primary sclerosing cholangitis (PSC) is a chronic, progressive liver disease characterised by biliary inflammation. Its exact aetiology remains unclear. This study aimed to use a combination of diagnostic methods and blood analysis to better understand the metabolic changes accompanying PSC. Methods: PSC patients (n = 60) and healthy controls (n = 60) were evaluated by magnetic resonance imaging and spectroscopy (hepatic fat and iron content), ultrasonography, standard blood analysis expanded with markers of iron metabolism, and colonoscopy. Results: PSC patients exhibited significant liver fibrosis and elevated liver enzymes. Serum hepcidin was increased (p < 0.01), but PSC patients did not show any markers of anaemia. Serum iron concentration and transferrin saturation were increased compared with healthy controls (p < 0.0001 and p < 0.001, respectively) but did not reach levels seen in hemochromatosis. In contrast, the liver iron content was lower in PSC patients (p < 0.0001). Conclusion: PSC patients demonstrated an atypical pattern of iron metabolism: elevated circulating iron and depleted liver iron stores despite increased hepcidin levels. This paradox suggests a disruption of hepcidin-mediated iron regulation, potentially sustaining a proinflammatory environment. Further investigation is warranted to clarify underlying mechanisms and therapeutic implications. Trial Registration: Clinical Trials I.D.: NCT06026449
SN  - 1932-6203
ER  -