A new seminar in The Lancet outlines the clinical profile of primary sclerosing cholangitis, a rare and chronic liver disease. The condition is characterized by inflammation and scarring of the bile ducts, leading to progressive liver damage. Its presentation is highly variable, but often involves slowly worsening fibrosis.
Inflammatory bowel disease co-occurs in a majority of patients, creating a complex clinical picture. The disease carries a significantly increased risk for developing cancers of the liver, bile ducts, and colon. This dual burden of chronic inflammation and cancer risk defines much of its management challenge.