Primary Sclerosing Cholangitis Differential Diagnoses

Updated: Nov 21, 2019
  • Author: Vikas Khurana, MD, FACP, FACG; Chief Editor: Vinay Kumar Kapoor, MBBS, MS, FRCS, FAMS  more...
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Diagnostic Considerations

The differential diagnosis of primary sclerosing cholangitis (PSC) includes congenital diseases (eg, Caroli disease, choledochal cysts) and secondary cholangiopathy, as observed in patients with collagen vascular diseases (eg, systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis) and in those with infiltrative diseases (eg, mediastinal fibrosis, Riedel thyroiditis, eosinophilic cholangitis, histiocytosis X). Infectious causes from parasitic, fungal, viral, or bacterial infections or from recurrent cholangitis itself, especially in patients who are immunocompromised, can cause multifocal liver abscesses that lead to a PSC-like appearance of the bile duct.

Biliary S100A9 levels may be a useful marker for PSC activity, and its implication in inflammation and carcinogenesis warrants further investigation. [28]

The most important differential diagnosis is from cholangiocarcinoma. CA 19-9 levels above 130 U/mL may suggest the diagnosis of cholangiocarcinoma, which can be established by brush cytology (endoscopic or percutaneous transhepatic).

Differential Diagnoses