Autoimmune hepatitis is a rare, long-term (chronic) disease of the liver. It happens when the body’s immune system causes damage to its own healthy liver cells. This leads to inflammation in the liver. AIH is more aggressive in children than in adults.

Types – Autoimmune hepatitis is split into two types. Each type has different autoantibodies present in the blood.

Type 1 (AIH-1)

This is the most common type of autoimmune liver disease in children. Two out of every three cases of AIH in children are type 1. This type usually starts in teenagers.

Type 2 (AIH-2)

This type of autoimmune liver disease is less common. It is more likely to affect young children, including infants. It is often more severe and gets worse (progresses) at a faster rate.

Treatment for AIH involves using medicines. They help make the immune system less active. If left untreated, AIH can cause serious scarring of the liver (cirrhosis). This could lead to liver failure in childhood or in adulthood. With treatment, the outlook for most children is very good.

Autoimmune sclerosing cholangitis is similar to AIH. You may hear it called juvenile sclerosing cholangitis or AIH/PSC overlap syndrome.

Children with ASC have inflammation of the liver (hepatitis). They have the same autoantibodies that are present in AIH-1. But they also have inflammation and infection of the bile ducts. This is known as cholangitis.

Autoimmune sclerosing cholangitis (ASC) =

autoimmune inflammation of the liver (AIH)
+
inflammation and infection of the bile ducts (cholangitis)

ASC affects boys and girls in equal numbers.

ASC is often linked with inflammatory bowel disease (IBD). The main types of IBD are Crohn’s disease and ulcerative colitis. They cause inflammation in the bowel (intestine). The main symptoms are severe tummy pain, diarrhoea and bloody poo (stools). Most cases of ASC are type 1. ASC is rarely linked with AIH-2.

This is a rare complication of liver transplant. It happens in around 2% to 6% of children who have a transplant for conditions other than autoimmune liver disease.

In AIH after liver transplant, the immune system attacks the new liver. The condition looks identical to autoimmune hepatitis. It is treated in the same way as this condition.

The cause of AIH after liver transplant is unclear. There may be a link to the medicines used after transplant. They may trigger an autoimmune reaction in a small number of children. It is important to treat AIH after transplant quickly to make sure that the new liver stays healthy.