Differentials
Obstructive bile duct lesion
SIGNS / SYMPTOMS
Previous history of bile duct surgery/obstruction.
Pain is much more prominent than with PBC.
Bacterial cholangitis as a complication is much more likely than in PBC.
INVESTIGATIONS
Antimitochondrial antibody or disease-specific antinuclear antibody not associated.
Abdominal ultrasound finding of duct dilatation (confirmed by magnetic resonance cholangiopancreatography [MRCP] or endoscopic retrograde cholangiopancreatography [ERCP] according to local practice) confirmatory.
Small-duct primary sclerosing cholangitis
SIGNS / SYMPTOMS
Different demographics than PBC (primary sclerosing cholangitis [PSC] is more common in younger males).
Association with inflammatory bowel disease.
INVESTIGATIONS
Antimitochondrial antibody or disease-specific antinuclear antibody not associated.
Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) suggestive of PSC may be present. This marker is absent in the majority of patients with PBC.
Abdominal ultrasound finding of duct irregularity (confirmed by magnetic resonance cholangiopancreatography [MRCP] or endoscopic retrograde cholangiopancreatography [ERCP] according to local practice) is confirmatory.
Liver biopsy characteristic of PSC.
Drug-induced cholestasis
SIGNS / SYMPTOMS
History of relevant drug exposure. Can also occur as a consequence of exposure to herbal/nonpharmaceutical preparations.
INVESTIGATIONS
Antimitochondrial antibody or disease-specific antinuclear antibody not associated.
Liver biopsy characteristic of cholestasis.
Cholestasis of pregnancy
SIGNS / SYMPTOMS
Associated with pregnancy.
INVESTIGATIONS
Antimitochondrial antibody or disease-specific antinuclear antibody not associated.
Bile acids elevated, transaminases more prominent than alkaline phosphatases.
Infiltrative malignancy within liver
SIGNS / SYMPTOMS
Associated with other systemic features suggestive of malignant disease.
INVESTIGATIONS
Cross-sectional imaging.
Liver biopsy if doubt persists.
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