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RESEARCH PRODUCT

Autoimmune hepatitis and overlap syndromes

Peter SchirmacherVolker DriesHeike ErberichAnsgar W. LohseHans Peter Dienes

subject

medicine.medical_specialtyCholangitis SclerosingAutoimmune hepatitisDiseasedigestive systemGastroenterologyPrimary sclerosing cholangitisLiver diseasePrimary biliary cirrhosisimmune system diseasesInternal medicinemedicineHumansAutoimmune liver diseaseAutoantibodiesHepatologyLiver Cirrhosis BiliaryBile ductbusiness.industryOverlap syndromemedicine.diseasedigestive system diseasesHepatitis Autoimmunemedicine.anatomical_structureLiverbusiness

description

Autoimmune hepatitis (AIH) is an immune-mediated, autodestructive liver disease with hepatocytes as target cells, mostly affecting young women. Primary biliary cirrhosis (PBC) is also regarded as an autoimmune liver disease with bile duct epithelia as the target cells, resulting in a continuous loss of bile ducts. Both diseases may occur simultaneously in their full manifestations in about 10% to 20% of cases, thus constituting an overlap syndrome with PBC directing the course of the disease. AIH may also occur simultaneously with primary sclerosing cholangitis (PSC), with a frequency of between 2% and 8% of patients with PSC. In most cases, AIH precedes manifestation of PSC. In children, the overlap syndrome of AIH and PSC seems to make up an entity of its own: autoimmune sclerosing cholangitis.

https://doi.org/10.1016/s1089-3261(02)00007-7