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Updated: Dec 14 2021

Primary Sclerosing Cholangitis

  • Snapshot
    • A 34-year-old man presents to his gastroenterologist's office complaining of worsening fatigue, jaundice, and pruritis over the last 2 months. He was diagnosed with ulcerative colitis 8 years ago. The physician ordered labs which came back with an elevated ALP, GGT, and bilirubin. She then scheduled the patient for magnetic resonance cholangiography and pancreatography which revealed multifocal stricturing of both the intrahepatic and extrahepatic biliary system. He is prescribed ursodeoxycholic acid.
  • Introduction
    • Clinical definition
      • primary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disorder characterized by multifocal biliary strictures and progressive liver disease
    • Genetics
      • significant genetic component but multifactorial
        • siblings at ↑ risk
    • Associated conditions
      • ulcerative colitis
  • Epidemiology
    • Demographics
      • young to middle-aged men
        • mean age of 30-40
    • Risk factors
      • inflammatory bowel disease (IBD)
        • primarily ulcerative colitis
        • up to 2/3 of PSC patients may have IBD
    • Pathogenesis
      • inflammation leads to multifocal fibrosis, sclerosis, and eventual stricturing and occlusion of both the extra- and intrahepatic bile ducts
        • the etiology of the inflammation is unknown
        • theorized to be caused by inflammatory mediators brought from the GI tract through the portal circulation
  • Presentation
    • Symptoms
      • usually insidious onset, presenting with
        • cholestatic symptoms
          • fatigue
          • jaundice
          • pruritis
        • +/- IBD symptoms
      • later, symptoms resulting from chronic bile stasis
        • steatorrhea
        • symptoms of fat-soluble vitamin deficiencies
        • episodes of cholangitis (RUQ pain and fever)
    • Physical exam
      • jaundice
      • signs of fat soluble vitamin deficiencies
      • in late disease
        • signs of cirrhosis and/or liver failure
          • e.g. ascites
  • imaging
    • Magnetic resonance cholangiography/pancreatography (MRCP)
      • first-line modality for diagnosis
      • shows segmental stricturing and dilation of the bile duct resulting in a "bead-like" appearance
    • Endoscopic retrograde cholangiography/pancreatography (ERCP)
      • gold standard
      • similar appearance as on MRCP
  • Studies
    • Diagnostic testing
      • diagnostic approach
        • requires a radiological diagnosis with exclusion of causes of secondary sclerosing cholangitis
      • studies
        • cholestatic liver labs
          • ↑ alkaline phosphatase (ALP) and ↑ GGT
          • +/- ↑ bilirubin
          • ALT and AST may be normal
        • p-ANCA
          • not specific nor required for diagnosis
        • liver biopsy
          • shows concentric periductal sclerosis (AKA "onion-skinning")
  • Differential
    • Primary biliary cirrhosis
      • commonly confused
      • distinguishing factors
        • typically no extrahepatic disease
        • typically female
        • positive anti-mitochondrial antibody (AMA)
    • Secondary cirrhosis
      • history of causative disease (e.g. alcohol use, hepatitis, etc.)
    • Primary cholangiocarcinoma
      • typically older
      • presence of mass without duct strictures on imaging
  • Treatment
    • Management approach
      • symptomatic treatment until liver transplant
      • no level 1 evidence of disease-altering treatments
    • First-line
      • ursodeoxycholic acid (cholestyramine)
        • relief of cholestatic symptoms (e.g., pruritus)
      • liver transplant
        • the only definitive/curative treatment
        • may recur even after transplant
    • Other treatments
      • endoscopic balloon dilatation and/or stenting
        • relieves symptoms via bile duct dilatation and improved bile flow
      • antibiotics
        • during episodes of ascending cholangitis
      • vitamin supplementation
  • Complications
    • Cirrhosis and end-stage liver disease
      • inevitable in the majority of patients
    • GI and hepatobiliary Mmalignancy
      • ↑↑ risk for cholangiocarcinoma
        • up to ~400x normal or around 10% of PSC patients
      • ↑ risk for colorectal cancer
        • ~5x risk
      • ↑ risk for several others including liver and gallbladder cancers
    • Ascending cholangitis
      • increased susceptibility because of bile stasis
    • Fat-soluble vitamin deficiencies
      • from the inability to release bile acid
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