Sclerosing cholangitis refers to chronic cholestatic syndromes characterized by patchy inflammation, fibrosis, and strictures of the intrahepatic and extrahepatic bile ducts. Progression obliterates the bile ducts and leads to cirrhosis, liver failure, and sometimes cholangiocarcinoma. Treatment is focused on surveillance for complications and endoscopic or surgical intervention for dominant strictures, as well as addressing the cause when possible.
(See also Overview of Biliary Function.)
Sclerosing cholangitis may be primary (with no known cause) or secondary. Causes of secondary sclerosing cholangitis include (1):
Infectious (eg, parasites, or opportunistic infections secondary to immune deficiency, as in HIV-related cholangiopathy)
Immune-mediated (eg, IgG4-related sclerosing cholangitis, transplant rejection)
Malignant (eg, cholangiocarcinoma or Langerhans cell histiocytosis, pancreatic cancer, metastatic disease)
Ischemic (eg, critical illness, thromboembolic disease)
Chemotherapy or medication induced (eg, intra-arterial floxuridine)Chemotherapy or medication induced (eg, intra-arterial floxuridine)
Obstructive (eg, choledocholithiasis, surgical stricture)
Both primary and secondary sclerosing cholangitis cause similar inflammatory and fibrosing lesions scarring the bile ducts.
Diagnosis of biliary strictures and dilation requires imaging techniques such as ultrasound, cholangiography, and CT.
Treatment focuses on relieving biliary obstruction (eg, dilating and stenting strictures) and, when possible, eradicating responsible organisms or treating the cause (eg, HIV).
Reference
1. Manns MP, Bergquist A, Karlsen TH, et al. Primary sclerosing cholangitis. Nat Rev Dis Primers. 2025;11(1):17. doi:10.1038/s41572-025-00600-x
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