Impact of age at diagnosis on disease progression in patients with primary sclerosing cholangitis

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 ¼ 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; p ¼ 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; p ¼ 0.003). Patients with late-onset PSC had reduced transplantation-free survival (10.5 AE 0.6 years vs. 20.8 AE 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.

Association between serum IgG level and clinical course in primary sclerosing cholangitis

“…We found that younger patients had significantly higher IgG levels and were a group that is at risk of shortened survival for this reason. In contrast to our findings, it has been previously reported that late-onset PSC and increased age in general are associated with poor survival promoted by the increased CCA risk over time [24–26]. However, our data underlines the fact that close surveillance is important in young patients with elevated IgG levels.…”

Effects of Primary Sclerosing Cholangitis on Risks of Cancer and Death in People With Inflammatory Bowel Disease, Based on Sex, Race, and Age

“…PSC affects all age groups, with older age at diagnosis reportedly associated with worse transplant-free survival. 1,6,8 However, causal mortality analysis according to age at PSC diagnosis has not been determined. Such findings have critical implications for interventional trial design, as 40 to 50% of all deaths may not be attributable to liver disease.…”

“…9,10 Phenotypic diversity according to sex and age may also influence the clinical course that patients experience. 8,11 Whilst the majority of datasets derive from Caucasian populations, a recent study indicates that PSC may be as common in patients of Afro-Caribbean descent. 12 However, it is unknown whether heritage-specific differences in disease risk exist in PSC.…”

Comprehensive Review of Molecular Mechanisms during Cholestatic Liver Injury and Cholangiocarcinoma

“…PSC is typically diagnosed in the third through fifth decades of life and has an approximately 3 to 1 male to female predilection. Diagnosis of PSC after the age of 50 is considered late onset and one study showed that late onset PSC was associated with more frequent dilatation therapy, recurrent cholangitis, and decreased transplant-free survival when compared to those diagnosed with PSC before age 50 [25]. PSC is a relatively rare disease and while the incidence is not well established, a systematic review of 8 studies from North America and Europe in 2011 showed an overall incidence of 0.77 per 100,000 person years [26].…”