Copyright ©The Author(s) 2023.
World J Hepatol. Apr 27, 2023; 15(4): 538-553
Published online Apr 27, 2023. doi: 10.4254/wjh.v15.i4.538
Table 1 Morphological classification of cholangiopathies
Pattern of biliary injury

CholangitisNeutrophilicInfection, sterile
LymphocyticPBC, GVHD, allograft rejection
PleomorphicAIH, PBC, DILI
GranulomatousPBC, sarcoidosis, DILI
SclerosingPSC, IgG4-sclerosing cholangitis, DILI
Bile duct loss and ductopeniaSee Table 2
Ductal plate malformationSee Table 3
NeoplasticBilIN, IPN, cholangiocarcinoma
Table 2 Conditions associated with ductopenia
Infantile and childhood diseasesBile duct paucity (syndromic/non-syndromic)
Extrahepatic biliary atresia
Progressive familial intrahepatic cholestasis
Fibropolycystic disease
Alpha-1 antitrypsin deficiency
Immune-mediated diseasesPrimary biliary cholangitis
Primary sclerosing cholangitis
Chronic graft-versus-host disease
Chronic hepatic allograft rejection
Hepatic sarcoidosis
Vascular diseasesIschaemic cholangiopathy
Portal biliopathy
Infectious diseasesAscending cholangitis
Protozoal and parasitic infestations
Drug- or toxin-induced biliary injury
Neoplastic diseasesHodgkin’s lymphoma
Langerhans cell histiocytosis
Systemic mastocytosis
IdiopathicIdiopathic adulthood ductopenia
Table 3 Ductal plate malformation – genes and encoded proteins
ADPKDPKD1Polycystin 1
PKD2Polycystin 2
GANAB/PKD3Glucosidase II alpha subunit
PCLDPRKCSHGlucosidase II beta subunit
SEC61BSEC61 translocon subunit beta
SEC63Translocon component in the endoplasmic reticulum
LRP5LDL receptor-related protein 5
CHF-SCZFYVE19Zinc finger FYVE-type containing 19