[Ahmed-USMLE]

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]

 

[khanar]

By the way this is a high yield syndrome
I've seen it everywhere in qbanks