Impact of Age at Diagnosis on Disease Progression in Patients with Primary Sclerosing Cholangitis

Overview

Journal United European Gastroenterol J

Publisher Wiley

Specialty Gastroenterology

Date 2018 Mar 8

PMID 29511555

Citations 10

Authors

Christian Rupp

Alexander Rossler

Taotao Zhou

Conrad Rauber

Kilian Friedrich

Andreas Wannhoff

Karl-Heinz Weiss

Peter Sauer

Peter Schirmacher

Caner Susal

Wolfgang Stremmel

Daniel N Gotthardt

Affiliations

Soon will be listed here.

Abstract

Background: The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30-40 years.

Objective: We aimed to analyse disease progression and liver-dependent survival in patients diagnosed with PSC after 50 years of age.

Methods: Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late-onset group.

Results: A total of 32/215 (14.9%) patients were diagnosed with PSC after 50 years of age. The proportion of females was significantly higher among patients with late-onset PSC (48.4 vs. 27.3%; = 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; = 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; = 0.003). Patients with late-onset PSC had reduced transplantation-free survival (10.5 ± 0.6 years vs. 20.8 ± 1.7 years, < 0.0001), with progredient liver failure and cholangiocarcinoma as the leading causes of death.

Conclusions: Patients with later age at diagnosis of PSC displayed a different clinical phenotype with a different sex ratio, immune status and an increased risk for progressive liver failure and biliary malignancies.

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References

Weismuller T, Trivedi P, Bergquist A, Imam M, Lenzen H, Ponsioen C . Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis. Gastroenterology. 2017; 152(8):1975-1984.e8. PMC: 5546611. DOI: 10.1053/j.gastro.2017.02.038. View

Meriggioli M, Sanders D . Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009; 8(5):475-90. PMC: 2730933. DOI: 10.1016/S1474-4422(09)70063-8. View

Sun X, Somada S, Shibata K, Muta H, Yamada H, Yoshihara H . A critical role of CD30 ligand/CD30 in controlling inflammatory bowel diseases in mice. Gastroenterology. 2008; 134(2):447-58. DOI: 10.1053/j.gastro.2007.11.004. View

Worthington J, Cullen S, Chapman R . Immunopathogenesis of primary sclerosing cholangitis. Clin Rev Allergy Immunol. 2005; 28(2):93-103. DOI: 10.1385/CRIAI:28:2:093. View

Amador-Patarroyo M, Rodriguez-Rodriguez A, Montoya-Ortiz G . How does age at onset influence the outcome of autoimmune diseases?. Autoimmune Dis. 2011; 2012:251730. PMC: 3238350. DOI: 10.1155/2012/251730. View

Boonstra K, Weersma R, van Erpecum K, Rauws E, Spanier B, Poen A . Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013; 58(6):2045-55. DOI: 10.1002/hep.26565. View

Lee Y, KAPLAN M . Primary sclerosing cholangitis. N Engl J Med. 1995; 332(14):924-33. DOI: 10.1056/NEJM199504063321406. View

Fattovich G, Vinante F, GIUSTINA G, Morosato L, Alberti A, Ruol A . Serum levels of soluble CD30 in chronic hepatitis B virus infection. Clin Exp Immunol. 1996; 103(1):105-10. PMC: 2200324. DOI: 10.1046/j.1365-2249.1996.915607.x. View

Lunder A, Hov J, Borthne A, Gleditsch J, Johannesen G, Tveit K . Prevalence of Sclerosing Cholangitis Detected by Magnetic Resonance Cholangiography in Patients With Long-term Inflammatory Bowel Disease. Gastroenterology. 2016; 151(4):660-669.e4. DOI: 10.1053/j.gastro.2016.06.021. View

10.

Papp M, Sipeki N, Vitalis Z, Tornai T, Altorjay I, Tornai I . High prevalence of IgA class anti-neutrophil cytoplasmic antibodies (ANCA) is associated with increased risk of bacterial infection in patients with cirrhosis. J Hepatol. 2013; 59(3):457-66. DOI: 10.1016/j.jhep.2013.04.018. View

11.

Tamir A, Eisenbraun M, Garcia G, Miller R . Age-dependent alterations in the assembly of signal transduction complexes at the site of T cell/APC interaction. J Immunol. 2000; 165(3):1243-51. DOI: 10.4049/jimmunol.165.3.1243. View

12.

Umeda K, Sun X, Guo Y, Yamada H, Shibata K, Yoshikai Y . Innate memory phenotype CD4+ T cells play a role in early protection against infection by Listeria monocytogenes in a CD30L-dependent manner. Microbiol Immunol. 2011; 55(9):645-56. DOI: 10.1111/j.1348-0421.2011.00362.x. View

13.

Ellinghaus D, Folseraas T, Holm K, Ellinghaus E, Melum E, Balschun T . Genome-wide association analysis in primary sclerosing cholangitis and ulcerative colitis identifies risk loci at GPR35 and TCF4. Hepatology. 2012; 58(3):1074-83. DOI: 10.1002/hep.25977. View

14.

Rupp C, Rossler A, Halibasic E, Sauer P, Weiss K, Friedrich K . Reduction in alkaline phosphatase is associated with longer survival in primary sclerosing cholangitis, independent of dominant stenosis. Aliment Pharmacol Ther. 2014; 40(11-12):1292-301. DOI: 10.1111/apt.12979. View

15.

Broome U, Olsson R, Loof L, Bodemar G, Hultcrantz R, Danielsson A . Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis. Gut. 1996; 38(4):610-5. PMC: 1383124. DOI: 10.1136/gut.38.4.610. View

16.

Salmi T, Collin P, Jarvinen O, Haimila K, Partanen J, Laurila K . Immunoglobulin A autoantibodies against transglutaminase 2 in the small intestinal mucosa predict forthcoming coeliac disease. Aliment Pharmacol Ther. 2006; 24(3):541-52. DOI: 10.1111/j.1365-2036.2006.02997.x. View

17.

Lamberts L, Janse M, Haagsma E, van den Berg A, Weersma R . Immune-mediated diseases in primary sclerosing cholangitis. Dig Liver Dis. 2011; 43(10):802-6. DOI: 10.1016/j.dld.2011.05.009. View

18.

Sebode M, Peiseler M, Franke B, Schwinge D, Schoknecht T, Wortmann F . Reduced FOXP3(+) regulatory T cells in patients with primary sclerosing cholangitis are associated with IL2RA gene polymorphisms. J Hepatol. 2014; 60(5):1010-6. DOI: 10.1016/j.jhep.2013.12.027. View

19.

Chrul S, Polakowska E . Age-dependent changes of serum soluble CD30 concentration in children. Pediatr Transplant. 2011; 15(5):515-8. DOI: 10.1111/j.1399-3046.2011.01516.x. View

20.

Gotthardt D, Rudolph G, Kloters-Plachky P, Kulaksiz H, Stiehl A . Endoscopic dilation of dominant stenoses in primary sclerosing cholangitis: outcome after long-term treatment. Gastrointest Endosc. 2010; 71(3):527-34. DOI: 10.1016/j.gie.2009.10.041. View