Autoimmune liver disorders in childhood include
autoimmune hepatitis and autoimmune
sclerosing cholangitis. These inflammatory liver disorders are characterised histologically by interface
hepatitis, biochemically by elevated
transaminase levels and serologically by
autoantibodies and increased levels of
immunoglobulin G.
Autoimmune hepatitis is particularly aggressive in children and progresses rapidly unless immunosuppressive treatment is started promptly. With appropriate treatment 80% of patients achieve remission and long-term survival. Autoimmune
sclerosing cholangitis responds to the same treatment used for
autoimmune hepatitis in regards to parenchymal
inflammation, but
bile duct disease progresses in about 50% of cases, leading to a worse prognosis and higher
transplantation requirement; it has a high recurrence rate post
liver transplant. New strategies aiming at treating unresponsive patients and at curbing specifically the liver autoimmune attack, without provoking unwanted systemic side effects, are being investigated and may be available within the next 5 years.