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%; p = 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; p = 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; p = 0.003). Patients with late-onset PSC had reduced transplantation-free survival (10.5 ± 0.6 years vs. 20.8 ± 1.7 years, p < 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.
Keywords: Primary sclerosing cholangitis; cholangiocarcinoma; dominant stenosis; immunosenescence; inflammatory bowel disease; liver transplantation.
The natural history of primary sclerosing cholangitis in 781 children: A multicenter, international collaboration.Hepatology. 2017 Aug;66(2):518-527. doi: 10.1002/hep.29204. Epub 2017 Jun 26. Hepatology. 2017. PMID: 28390159 Clinical Trial.
Epidemiology and outcomes of primary sclerosing cholangitis with and without inflammatory bowel disease in an Australian cohort.Liver Int. 2017 Mar;37(3):442-448. doi: 10.1111/liv.13328. Epub 2017 Jan 24. Liver Int. 2017. PMID: 27891750 Clinical Trial.
Primary sclerosing cholangitis.Orphanet J Rare Dis. 2006 Oct 24;1:41. doi: 10.1186/1750-1172-1-41. Orphanet J Rare Dis. 2006. PMID: 17062136 Free PMC article.
Primary sclerosing cholangitis: diagnosis, prognosis, and management.Clin Gastroenterol Hepatol. 2013 Aug;11(8):898-907. doi: 10.1016/j.cgh.2013.02.016. Epub 2013 Feb 27. Clin Gastroenterol Hepatol. 2013. PMID: 23454027 Free PMC article. Review.
Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease.Dig Dis. 2012;30(2):216-9. doi: 10.1159/000336706. Epub 2012 Jun 20. Dig Dis. 2012. PMID: 22722442 Review.
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