Gastrointestinal System >
Auto-Immune Hepatobiliary Disease

“Autoimmune diseases of the liver and biliary system”

  • Three diseases include:
    • Autoimmune Hepatitis (AIH)
    • Primary Biliary Cirrhosis/Cholangitis (PBC)
    • Primary Sclerosing Cholangitis (PSC)
Risk Factors
  • AIH:
    • Typically common in young women
    • Autoimmune conditions
  • PBC:
    • Common in middle-aged women with recurrent UTIs
    • +ve family history
    • Smoking
    • Past pregnancy
  • PSC:
    • Common among middle-aged men with ulcerative colitis
Aetiology
  • Generally idiopathic
  • Commonly associated with other autoimmune conditions – T1DM, Hashimoto’s thyroiditis, coeliac disease
  • Genetic predisposition
  • Possible environmental triggers
Pathophysiology
  • AIH:
    • Abnormal T cell function and autoantibodies directed against hepatocyte surface antigens.
    • Classification by autoantibodies
  • PBC:
    1. Inflammation and the progressive destruction of only intrahepatic bile ducts
    2. Defective bile duct regeneration
    3. Chronic cholestasis
    4. Fibrotic changes
    5. Liver failure
  • PSC:
    • Progressive chronic inflammation of intrahepatic and extrahepatic bile ducts
Clinical Presentation
  • AIH:
    • Acute hepatitis and signs of autoimmune disease – Fatigue, abdominal pain, weight loss, jaundice, RUQ pain, fever
  • PBC:
    • Lethargy, sleepiness, and pruritis may precede jaundice by years.
    • Skin pigmentation
    • Xanthomata
    • Hepatosplenomegaly
  • PSC:
    • Incidental ↑ ­ALP finding in patient with IBD, pruritis, fatigue, jaundice, hepatomegaly
Investigations
  • AIH:
    • ­↑ Serum bilirubin, ­ ↑ AST, ­↑ ALT and ­↑ ALP, hypergammaglobulinaemia, +ve autoantibodies (ASMA, ANA, ­↑ IgG, LKM1). Anaemia, ↓ WCC and ↓ platelets indicate hypersplenism
    • Liver biopsy (mononuclear infiltrate of the portal and periportal areas
  • PBC:
    • ­↑ ALP, ↑ ­GGT, ↑­bilirubin, ↓ albumin, ­↑ prothrombin time. 98% are AMA M2 subtype +ve.
    • Ultrasound to exclude extrahepatic cholestasis
  • PSC:
    • ­↑ ALP, ↑ ­bilirubin, hypergammaglobulinaemia, ↑ ­IgM, AMA -ve but ANA, SMA and ANCA maybe +ve.
    • ERCP or MRCP to reveal duct anatomy and damage.
    • Liver biopsy shows fibrous obliterative damage
Management
  • AIH:
    • Immunosuppressant therapy
      • Prednisolone30mg/d PO for 1 month, ↓ by 5mg a month to a maintenance dose of 5-10mg/d PO.
      • Azathioprine (50-100mg/d PO) used as a steroid-sparing agent to maintain remission.
    • Liver Transplantation in decompensated cirrhosis or failure to medical therapy
  • PBC:
    • Colestyramine for pruritis, osteoporosis prevention, Fat-soluble vitamin prophylaxis (vitamin A, D and K), consider high dose ursodeoxycholic acid to improve survival and delay transplantation.
    • Regular monitoring
  • PSC:
    • Liver transplantation is the mainstay for end-stage disease, recurrence occurs in up to 30%; 5% graft survival is >60%
Complications
  • Liver cirrhosis and portal hypertension
  • Osteoporosis
  • Malabsorption.
  • Cholangiocarcinoma
  • Increased risk of hepatocellular, colorectal, pancreatic and gallbladder cancer

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