Primary sclerosing cholangitis

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Epidemiology

Tip

Compared with Primary biliary cholangitis, which are common among middle-aged women.

The exact cause is unknown.
Associations
- Chronic inflammatory bowel diseases (IBD)
  - ∼ 90% of patients with PSC have IBD (approx. 87% of these patients have ulcerative colitis)
- Presence of HLA-B8 and HLA-DR3

Tip

The majority of patients with PSC also have ulcerative colitis.

Progressive chronic inflammation of both intrahepatic and extrahepatic bile ducts

Signs of cholestasis
- Jaundice/scleral icterus
- Pruritus
- Pale stool, dark urine
- Fatigue
- Can lead to acute cholangitis (fever, chills, right upper quadrant pain)
Later stages: signs of cirrhosis
- Hepatosplenomegaly
- Portal hypertension
- Liver failure
Symptoms of chronic inflammatory bowel disease, which is frequently associated with PSC, or other associated comorbidities

Mnemonic

Suspect PSC in patients with a history of inflammatory bowel disease and elevated cholestatic enzymes (ALP, GGT, and conjugated bilirubin).

Typical perinuclear anti-neutrophil cytoplasmic antibodies (pANCA): present in up to 80% of patients with PSC.

Method of choice (if there is no biliary obstruction)
Supportive findings: multifocal intrahepatic and extrahepatic bile duct strictures alternating with dilatation and beading of bile ducts There is irregular dilatation (green overlay) of intrahepatic bile ducts proximal to a common duct stenosis (arrow). Alternation of strictured and dilated segments creates a beaded appearance. Peripheral ducts appear pruned as a result of obliteration (examples indicated by arrowheads), and scattered biliary diverticula are seen (example indicated by red overlay).