Overview
Definition
A chronic, progressive cholestatic liver disease characterized by inflammation, obliterative fibrosis, and stricturing of both intrahepatic and extrahepatic bile ducts.
Epidemiology
More common in males (2:1 ratio) around 30-50 years of age. Strongly associated with Inflammatory Bowel Disease (especially Ulcerative Colitis in >70-80% of cases).
Etiology & Risk Factors
- T-cell mediated autoimmune destruction of biliary tree
- Strong association with HLA alleles and gut dysbiosis
Clinical Symptoms
- Fatigue
- Pruritus
- Jaundice
- Right upper quadrant abdominal pain
- Recurrent episodes of ascending cholangitis
Clinical Approach
Diagnosis
- Magnetic Resonance Cholangiopancreatography (MRCP) showing classic 'beaded' appearance of bile ducts (segmental strictures and dilations)
- Elevated ALP
- p-ANCA positivity (common but non-specific)
Management
- Endoscopic retrograde cholangiopancreatography (ERCP) with balloon dilation for dominant strictures
- Ursodeoxycholic acid (controversial benefit)
- Liver transplantation (definitive)
Complications
- Cholangiocarcinoma (annual screen required)
- Liver cirrhosis / Portal hypertension
- Recurrent bacterial cholangitis
- Colorectal cancer (due to associated UC)