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, 6 (2), 255-262

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

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

Christian Rupp et al. United European Gastroenterol J.

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%; 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.

Figures

Figure 1.
Figure 1.
Distribution of age at first diagnosis of PSC. Distribution of age at first diagnosis of PSC differentiate into decades for the whole study cohort (N = 215) The number of patients is plotted on the y-axis. The sex ratio in patients with diagnosis after the age of 50 is significantly different compared with patients with earlier onset.
Figure 2.
Figure 2.
Transplantation-free survival in patients with early- and late-onset PSC. Actuarial transplantation-free survival (Kaplan–Meier estimate) of patients with early- or late-onset of PSC. Time is given in years from the time of first diagnosis (x-axis). The percentage of patients surviving without liver transplantation is plotted on the y-axis. Patients with late-onset PSC showed significantly lower transplantation-free survival.

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