Article Sections

Introduction

Primary sclerosing cholangitis (PSC) is a chronic, progressive biliary disease characterized by fibrosis of the bile ducts.  It primarily affects young men and is associated with significant morbidity and mortality.

Pathophysiology

PSC is a chronic disease characterized by inflammation and fibrosis of medium and large intrahepatic and/or extrahepatic bile ducts, resulting in multifocal segmental strictures.  Histologic features include fibrous obliteration of the bile ducts and concentric periductal connective tissue deposition, the latter of which resembles an onion skin-like pattern ( Image 1).  Bile duct fibrosis causes biliary obstruction and ultimately cirrhosis.

Epidemiology

The condition predominantly affects young men (age 30-40).  Up to 90% of patients with PSC also have inflammatory bowel disease (IBD), especially ulcerative colitis.  Conversely, 5% of patients with IBD have PSC, higher than in the general population.

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