What happens if the disease gets worse over time?

A small number of children will not respond to treatment. If treatments are not effective, damage to the liver can become worse over time.

In ASC, bile duct disease gets worse over time in about half of children.

Complications of long-term (chronic) liver disease include:

• weight loss or poor weight gain
• fluid in the tummy (ascites)
• high blood pressure in the blood vessels around the liver (portal hypertension)
• yellowing of the whites of the eyes and skin
• itchy skin (pruritus)
• bile duct infection (cholangitis)

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Liver transplant

Liver transplant is only considered if children don’t respond to medicines and the liver is damaged beyond repair. The rate of liver transplant is higher in children with ASC.

A liver transplant is an operation to remove a liver that does not work. It is replaced with a healthy liver from another person (donor). A successful liver transplant will help a child survive when the liver is failing. It will also significantly improve a child’s quality of life.

A liver transplant is a major operation. It will only be done after the benefits and risks have been carefully weighed up. Following a successful liver transplant children need lifelong medicines and follow up.

Find out more about liver transplant

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Autoimmune liver disease that comes back after transplant

Autoimmune liver disease can come back in the new liver following transplant. Doctors called this recurrence. It is different to AIH following transplant for a different type of liver disease (de novo AIH).

If autoimmune hepatitis comes back, treatment for AIH will be re-introduced. Children usually respond well to treatment. But some research suggests that children with AIH type 1 have a better outcome than those with AIH type 2. It is important to treat AIH after transplant quickly. This helps the new liver stay healthy.

Autoimmune sclerosing cholangitis can also come back after transplant. This is often accompanied by inflammatory bowel disease (IBD). This may get worse over time (progress). There are no standard treatments for ASC after transplant. Up to two thirds of children will eventually need another liver transplant.

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