skip to main content

Primary biliary cholangitis — ESEGH MCQ

Instant feedback + full explanation. One question, done properly.

HardHepatologyPrimary biliary cholangitisESEGH

A 46-year-old woman is referred with 9 months of pruritus and fatigue. Blood tests show ALP 386 IU/L (upper limit of normal 120), GGT 412 IU/L, ALT 58 IU/L (upper limit of normal 40), bilirubin 18 micromol/L, raised IgM and normal IgG. Ultrasound and MRCP show no biliary dilatation or stricturing. Antimitochondrial antibody is negative by immunofluorescence and anti-M2 ELISA, but antinuclear antibody testing shows a nuclear-rim pattern with anti-gp210 reactivity. She has no inflammatory bowel disease and has taken no potentially cholestatic medication. What is the most likely diagnosis?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: E — Primary biliary cholangitis

This is antimitochondrial-antibody-negative primary biliary cholangitis. The discriminating features are persistent cholestatic biochemistry, pruritus, raised IgM, normal biliary imaging and the PBC-specific nuclear-rim anti-gp210 antibody. UK guidance accepts highly PBC-specific ANA, in the appropriate cholestatic context and without another explanation, as sufficient for diagnosis; routine diagnostic liver biopsy is unnecessary. Small-duct PSC can have a normal MRCP but does not explain the PBC-specific serology and would generally require histological confirmation. IgG4-related sclerosing cholangitis usually produces biliary stricturing and often accompanies raised IgG4 or autoimmune pancreatitis. Drug-induced cholestasis requires a compatible exposure. Autoimmune hepatitis usually causes a predominantly hepatitic pattern with raised IgG; the mild ALT elevation here does not establish a PBC–AIH variant.

Reference: Hirschfield GM et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines, Recommendations 2, 4 and 5, 2018. https://www.bsg.org.uk/getattachment/de43e6dc-ebd3-4145-82de-724db091f544/BSG-and-UKPBC-primary-biliary-cholangitis-treatment-and-management-guidelines.pdf%3Flang%3Den-US