Elevated long-to-very-long-chain ceramide ratio correlates with disease severity in inflammatory bowel disease and primary sclerosing cholangitis

Elger, Tanja and Huss, Muriel and Liebisch, Gerhard and Hoering, Marcus and Loibl, Johanna and Kandulski, Arne and Mueller, Martina and Tews, Hauke Christian and Buechler, Christa (2025) Elevated long-to-very-long-chain ceramide ratio correlates with disease severity in inflammatory bowel disease and primary sclerosing cholangitis. SCIENTIFIC REPORTS, 15 (1): 20294. ISSN 2045-2322,

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Abstract

There is strong evidence that ceramides play a significant role in the pathology of inflammatory bowel disease (IBD) and chronic liver injury. Long-chain (LC) and very long-chain (VLC) ceramides have opposing functions, yet the associations of circulating levels of ceramide species in patients with IBD and primary sclerosing cholangitis (PSC)-as inflammatory biliary-hepatic disease closely linked to IBD- with disease severity remain poorly studied. This study investigates whether serum levels of ceramide (Cer) and hexosylceramide, a glycated ceramide derivative, are associated with disease severity in these conditions. Serum levels of eight ceramide and five hexosylceramide species were measured in 16 healthy controls, 57 patients with IBD, 7 patients with PSC, and 13 patients with PSC-IBD. Lipid levels were determined using direct flow injection analysis with a triple quadrupole mass spectrometer. Patients with IBD exhibited higher levels of Cer 18:1;O2/16:0 and Cer 18:1;O2/18:0 compared to controls. Their LC/VLC ceramide ratio was elevated and positively correlated with C-reactive protein and fecal calprotectin. However, ceramide and hexosylceramide levels were not associated with stool consistency, disease localization, or extra-intestinal manifestations. Patients with PSC and PSC-IBD also had increased LC/VLC ceramide ratios, primarily due to a decline in VLC ceramide species. In PSC-IBD, this ratio correlated positively with cholestasis markers. Additionally, serum hexosylceramide 18:1;O2/16:0 and 24:1 levels were specifically elevated in PSC. This study demonstrates that an altered LC/VLC ceramide balance is associated with disease severity in IBD, PSC-IBD, and PSC, highlighting its potential as a biomarker for IBD, PSC-IBD, and PSC. As our PSC cohorts were small, a confirmatory study is required.

Item Type: Article
Uncontrolled Keywords: COLITIS; CELL; METABOLISM; UPDATE; Calprotectin; Inflammatory bowel disease; Disease activity; Primary sclerosing cholangitis
Subjects: 600 Technology > 610 Medical sciences Medicine
Divisions: Medicine > Lehrstuhl für Innere Medizin I
Medicine > Lehrstuhl für Klinische Chemie und Laboratoriumsmedizin
Depositing User: Dr. Gernot Deinzer
Date Deposited: 26 Aug 2026 09:34
Last Modified: 26 Aug 2026 09:34
URI: https://pred.uni-regensburg.de/id/eprint/66079

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