Autoimmune Liver Disease in Children

Types of Autoimmune liver diseases:

Autoimmune hepatitis (AIH) and AIH/sclerosing cholangitis overlap syndrome known as autoimmune sclerosing cholangitis (ASC)

What is autoimmune hepatitis?

Hepatitis means inflammation of the liver. In autoimmune hepatitis, live cells or hepatocytes are the target of the immune system causing inflammation of the liver cells. This type of hepatitis is different than the hepatitis caused by a virus like hepatitis A, B and C.

What is autoimmune sclerosing cholangitis?

Autoimmune sclerosing cholangitis and autoimmune hepatitis are similar, but along with hepatitis, there is also inflammation of the bile ducts. Bile ducts are responsible for draining bile out of the liver.

Most common symptoms:

  • Tiredness and generally feeling unwell
  • Loss of appetite

Other symptoms:

  • Nausea or being sick
  • Abdominal pain
  • Jaundice with dark urine and pale stool
  • Joint and muscle pain
  • Loss of appetite and weight loss
  • Itching
  • Fever
  • Nosebleeds bleeding gums and bruising easily
  • Amenorrhea (delayed starting of periods or they stop once started)
  • Diarrhea (bowel symptoms are more common in autoimmune sclerosing cholangitis)

Later symptoms:

  • Swollen abdomen
  • Swelling, especially in lower legs
  • Irritability or confusion

How is it diagnosed?

  • Blood tests
  • Liver biopsy
  • Ultrasound
  • MRI-MRCP scan
  • Gastrointestinal endoscopy if having bowel symptoms


  • Steroids (prednisolone)
  • Medications (Cellcept and Azathioprine)
  • Treatment will be ongoing, and the child may need to be on a combination of medications for years
  • Autoimmune liver disease is monitored by a specialist through regular blood tests

Last updated on August 15th, 2023 at 12:30 pm

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