The variant forms of cholestatic diseases involving small bile ducts in adults

Abstract

SEASES of the biliary system can be subdivided into small-duct diseases which require liver biopsy to establish the diagnosis and large-duct diseases which are diagnosed by cholangiography or other imaging studies. The term “small-ducts” refers to interlobular and proximal septal bile ducts. Small-duct diseases in adults can be classified according to etiology: 1) part of a systemic condition, including sarcoidosis, cystic fibrosis, graft-versus-host disease and malignancy (e.g. Hodgkin’s disease); 2) specific etiology including toxic or ischemic damage and rejection of transplant liver; and 3) primary diseases including primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and idiopathic adulthood ductopenia. The majority of adult patients with chronic cholestasis have PBC or PSC. These diseases have been extensively described in their typical forms. However, recent data indicate that subsets of patients must be individualized in terms of diagnostic criteria or management. In this paper, we review the variants forms of the primary small-duct diseases in adults (Table 1).