Autoimmune Liver Disease in Children
Overview:
Autoimmune hepatitis (AIH) and 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 differs from 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.
Symptoms and Causes:
The cause of autoimmune hepatitis in children is unknown. Factors may include genetics or general issues with the immune system.
Symptoms may include:
- Tiredness and generally feeling unwell
- Loss of appetite
- 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
- Change in sleep patterns
Diagnosis and Tests:
- Blood tests
- Liver biopsy
- Ultrasound
- MRI-MRCP scan
- Gastrointestinal endoscopy if having bowel symptoms
Management and Treatment
- Steroids (prednisolone/prednisone)
- 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
Prevention
There is no known way to prevent autoimmune liver disease in children.
Outlook and Prognosis
Most cases of autoimmune liver disease can be controlled with medication. In some rare cases a liver transplant may be needed.
Living With
- Be sure your child has regular medical check-ups and takes medications as prescribed.
- Be mindful of your child’s diet. If necessary, consult with a pediatric dietitian.
- Inquire if exercise may be beneficial and be mindful of activities your child should avoid.
Medically Reviewed March 2025
Last updated on March 18th, 2025 at 02:01 pm