<aside> 💡 Persistent, progressive, biliary Inflammation & obliterative fibrosis of all bile ducts both intra & extra hepatic. Small, medium and large.

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Demographics

• 30-50y, males more common 2:1.

Aetiology

Idiopathic

Risk Factors

• Associated with other fibrosing disorders (IgG4 related disorders, autoimmune pancreatitis)
• IBD particularly ulcerative colitis in 70% patients.
• Family history, HLA-B8, perinuclear pANCA,

Pathophysiology

• Genetic and environmental factors impact liver/gut cross communication through intestinal microbiome, epigenome and metabolome.
• leads to constriction and inflammation of intra and extrahepatic bile ducts
• outcomes range from chronic liver cholestasis and inflammation to malignant transformation of the liver and gut and to metabolic bone disease