Associated with brain damage. Etiology is unknown. Histologically, presents in the form of giant cell hepatitis. An autoimmune disease characterized by destruction of intrahepatic bile ducts and obstructive jaundice. Typically affects middle aged women.
Antimitochondrial antibodies are present in the serum of almost all patients.
Disease progresses to cirrhosis . A chronic inflammatory disease of extrahepatic bile ducts. It causes obstructive jaundice and biliary cirrhosis . Acute disease of children characterized by hepatic failure and encephalopathy. Histologically, the liver shows microvesicular steatosis.