TABLE 1.
TABLE. Classification of the Cholangiopathiesa
| Genetic |
| Alagille syndrome |
| Caroli syndrome |
| Cystic fibrosis |
| Polycystic liver disease |
| ADPLD |
| ADPKD |
| ARPKD |
|
|
| Idiopathic |
| Autoimmune cholangitis |
| Biliary atresiab |
| Idiopathic childhood/adulthood ductopenia |
| IgG4-associated cholangitis |
| Primary biliary cirrhosisb |
| Primary sclerosing cholangitisb |
|
|
| Malignant |
| Cholangiocarcinoma |
|
|
| Secondary sclerosing cholangitis |
| ABCB4 deficiency |
| Abdominal trauma (surgical or blunt) |
| AIDS cholangiopathy |
| Amyloidosis |
| Chemical/drugs (ie, 5-fluorouracil) |
| Choledocholithiasis |
| Eosinophilic or mast cell cholangitis |
| Graft-vs-host disease involving the liver |
| Iatrogenic biliary strictures |
| Portal hypertensive biliopathy |
| Recurrent pyogenic cholangitis |
| Sarcoidosis |
| Sickle cell disease |
| Vascular/ischemic (ie, hepatic artery stenosis after liver transplant) |
a
ADPLD = autosomal dominant polycystic liver disease; ADPKD = autosomal dominant polycystic kidney disease; AIDS = acquired immunodeficiency syndrome; ARPKD = autosomal recessive polycystic kidney disease.
b
For the genetic component of these diseases, see Supplemental Table 2.