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. Author manuscript; available in PMC: 2017 Aug 11.
Published in final edited form as: N Engl J Med. 2016 Sep 22;375(12):1161–1170. doi: 10.1056/NEJMra1506330

Table 1.

Causes of Secondary Sclerosing Cholangitis.*

Cause Proposed Pathogenesis
Abdominal trauma Damage and subsequent strictures of the biliary tree
AIDS-related cholangiopathy Biliary strictures associated with infection, most commonly due to Cryptosporidium parvum
Amyloidosis Systemic disease involving the biliary tree
Cholangiocarcinoma New development of cancer that mimics clinical presentation of primary sclerosing cholangitis
Choledocholithiasis Strictures due to a stone or stones within the biliary tree
Eosinophilic cholangiopathy Systemic disease involving the biliary tree
Graft-versus-host disease Systemic disease involving the biliary tree
Hepatic inflammatory pseudotumor Inflammatory condition that mimics cholangiographic features of primary sclerosing cholangitis
Histiocytosis X Systemic disease involving the biliary tree
Iatrogenic biliary strictures Strictures due to surgery or ERCP
IgG4-associated cholangitis Systemic disorder that is characterized by high serum IgG4 levels and IgG4-positive lymphoplasmacytic infiltration of affected organs (the pancreas and bile ducts) and that causes biliary strictures
Intraarterial chemotherapy Biliary strictures due to infusion offluorouracil chemotherapy through the hepatic artery
Ischemic cholangiopathy Inadequate arterial supply of the biliary tree
Mast-cell cholangiopathy Systemic disease involving the biliary tree
Portal hypertensive biliopathy Extrahepatic portal venous obstruction causing compression and stricturing of the biliary tree
Recurrent pyogenic cholangitis Progressive and diffuse biliary stricturing, ectasias, and local stone formation; common in East Asia
Sarcoidosis Systemic disease involving the biliary tree
*

AIDS denotes acquired immunodeficiency syndrome, and ERCP endoscopic retrograde cholangiopancreatography.