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Caroli disease is unlike Primary Biliary Cirrhosis is a Congenital dilatation of the intra hepatic biliary ducts has nothing to do with autoimmunity.
Caroli syndrome is Caroli disease + Congenital hepatic fibrosis + Polycystic kidney disease [infantile or adult]
Caroli syndrome is Caroli disease + Congenital hepatic fibrosis + Polycystic kidney disease [infantile or adult]