Primary sclerosing cholangitis
Introduction
Primary sclerosing cholangitis is a biliary disease of unknown aetiology characterised by inflammation and fibrosis of intra and extra-hepatic bile ducts.
Epidemiology
- Incidence: 2.00 cases per 100,000 person-years
- Peak incidence: 40-50 years
- Sex ratio: more common in males 2:1
| Condition | Relative incidence |
|---|---|
| Primary biliary cholangitis | 6.00 |
| Primary sclerosing cholangitis | 1 |
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Aetiology
Associations
- ulcerative colitis: 4% of patients with UC have PSC, 80% of patients with PSC have UC
- Crohn's (much less common association than UC)
- HIV
Clinical features
Features
- cholestasis: jaundice and pruritus
- right upper quadrant pain
- fatigue
Investigations
Investigation
- endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) are the standard diagnostic investigations, showing multiple biliary strictures giving a 'beaded' appearance
- ANCA may be positive
- there is a limited role for liver biopsy, which may show fibrous, obliterative cholangitis often described as 'onion skin'
Complications
Complications
- cholangiocarcinoma (in 10%)
- increased risk of colorectal cancer