Table 1.

Classification of cholangiopathies.

Genetic
Alagille's syndrome
Caroli's syndrome
Cystic fibrosis
MDR3 deficiency
Polycystic liver disease (ADPLD, ADPKD, ARPKD)
Immune-mediated
Acute allograft rejection
Chronic allograft rejection
Graft versus host disease
Primary biliary cholangitis
Idiopathic
Biliary atresia
Idiopathic childhood/adulthood ductopenia
IgG4 cholangiopathy
Primary sclerosing cholangitis
Sarcoidosis
Infectious
AIDS cholangiopathy (e.g., viral cholangitis)
Bacterial cholangitis (e.g., Escherichia coli, Klebsiella, Enterococcus, Enterobacter, Pseudomonas, anaerobes)
Parasitic cholangitis (e.g., Ascaris lumbricoides, Opisthorchis viverrini, Clonorchis sinensis, Fasciola hepatica)
Malignant
Cholangiocarcinoma
Other
Drug-induced (e.g., Floxuridine-induced cholangiopathy, ketamine cholangiopathy)
Vascular/ischemic (e.g., post-liver transplant hepatic artery stenosis, systemic vasculitis)

Abbreviations: ADPKD, autosomal dominant polycystic kidney disease; ADPLD, autosomal dominant polycystic liver disease; AIDS, acquired immunodeficiency syndrome; ARPKD, autosomal recessive polycystic kidney disease; IgG4, immunoglobulin G subclass 4; MDR3, multidrug resistance 3.