• Clinical science

Primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis (PSC) is a progressive chronic inflammation of both the intrahepatic and extrahepatic bile ducts. While the exact etiology is unknown, there is a strong association with autoimmune diseases, particularly ulcerative colitis (UC). In the early stages, PSC is usually asymptomatic. Later in the course of the disease, patients present with symptoms of cholestasis (e.g., pruritus, jaundice). Laboratory abnormalities include elevated liver function tests and autoantibodies (pANCA in up to 80% of cases). Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (ERCP) is performed to confirm the diagnosis. Management is primarily symptomatic, with liver transplantation reserved for end-stage liver disease.

Epidemiology

  • Sex: > (2:1)
  • Age: The median age at diagnosis is ∼ 40.

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Associations
    • Chronic inflammatory bowel diseases (IBD)
    • HLA-B8 and HLA-DR3
    • Other autoimmune conditions (e.g., hypergammaglobulinemia IgM)

The majority of PSC patients also have ulcerative colitis! References:[2][3][1][4]

Clinical features

References:[2][3]

Diagnostics

Laboratory findings

Imaging

  • Cholangiography
    • Method of choice: magnetic resonance cholangiopancreatography (MRCP)
    • Alternatives
      • Endoscopic retrograde cholangiopancreatography (ERCP)
        • More invasive but also more accurate than MRCP
        • Good alternative for patients who cannot undergo MRI testing (e.g., patients with pacemaker)
      • Percutaneous transhepatic cholangiography (PTC)
        • Most invasive test
        • For patients who cannot undergo ERCP
    • Findings: multifocal strictures alternating with dilation and beading of bile ducts
  • Ultrasound
    • Irregular diameter of the bile duct
    • Diffuse thickening of the wall of the common hepatic and bile ducts
  • Following diagnosis → colonoscopy (to assess for UC)

Liver biopsy

  • Not an essential part of the workup; usually done if small duct PSC is suspected, which is not always detectable via cholangiography
  • Typical finding: "onion skin" scarring and fibrosis of bile ducts
  • Antibiotics (e.g., ciprofloxacin) can be given before the procedure to decrease the risk of developing consecutive cholangitis.

If a patient with pre-existing chronic inflammatory bowel disease displays increased ALP, GGT, and conjugated bilirubin, always consider PSC!References:[1][3][2][4]

Pathology

Differential diagnoses

Differential diagnoses of primary cholangitis
Primary sclerosing cholangitis Primary biliary cholangitis
Epidemiology
  • More common among middle-aged men

  • More common among middle-aged women

Pathophysiology
  • Progressive chronic inflammation of both intrahepatic and extrahepatic bile ducts

  • Progressive destruction of only intrahepatic small and medium-sized bile ducts

Clinical presentation
Laboratory tests
Complications
  • Associated with autoimmune conditions

The differential diagnoses listed here are not exhaustive.

Treatment

References:[5][6][7][8][3]

Complications

References:[2]

We list the most important complications. The selection is not exhaustive.

Prognosis

References:[9]

last updated 09/25/2018
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