Variations in primary sclerosing cholangitis across the age spectrum

Area of Science:

• Gastroenterology
• Hepatology
• Cholangiopathies

Background:

• Primary sclerosing cholangitis (PSC) is a chronic liver disease typically diagnosed in middle-aged adults.
• Limited data exists on how age at diagnosis influences PSC presentation and outcomes.
• Understanding age-related phenotypic differences is crucial for personalized patient management.

Purpose of the Study:

• To compare the clinical characteristics and outcomes of PSC based on the age of diagnosis.
• To identify potential differences in disease progression and complications across different age groups.

Main Methods:

• A multicenter retrospective review of a large primary sclerosing cholangitis cohort.
• Patients were categorized into three age groups at diagnosis: 1-19 years (early diagnosis, ED), 20-59 years (middle-age diagnosis, MD), and 60-79 years (late diagnosis, LD).
• Clinical features, including small-duct PSC, hepatobiliary malignancies, and hepatic decompensation, were analyzed.

Main Results:

• Early diagnosis (ED) of PSC was associated with a higher prevalence of small-duct PSC (13%) compared to middle-age (MD) (5%) and late diagnosis (LD) (2%) groups.
• Patients with ED showed a reduced risk of hepatobiliary malignancies.
• Late diagnosis (LD) of PSC was linked to a higher likelihood of cholangiocarcinoma diagnosis within one year of PSC diagnosis and increased risk of hepatic decompensation.

Conclusions:

• Individuals diagnosed with PSC early in life tend to have small-duct PSC and experience fewer disease-related complications.
• Clinicians should maintain a high index of suspicion for cholangiocarcinoma in patients diagnosed with PSC late in life.
• Age at diagnosis is a significant factor influencing PSC phenotype and clinical outcomes.