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AIH Mimicking Rheumatic — SCE Rheumatology MCQ

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ModerateAutoimmune SerologyAIH Mimicking RheumaticSCE Rheumatology

A 45-year-old woman is referred to rheumatology with fatigue and intermittent arthralgia affecting her hands and knees. There is no synovitis, rash, photosensitivity, oral ulceration, sicca syndrome or Raynaud phenomenon. Investigations show ANA 1:320 with a homogeneous pattern, smooth-muscle antibodies 1:160, negative anti-dsDNA and ENA, and normal C3 and C4. ALT is 486 U/L, AST 371 U/L, ALP 138 U/L (upper limit of normal 120 U/L), bilirubin 18 micromol/L and IgG 25 g/L (reference range 6–16 g/L). Viral hepatitis has been excluded, and there is no relevant medication, supplement or alcohol exposure. Which diagnosis most likely explains the presentation?

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Correct answer: C — Type 1 autoimmune hepatitis

Type 1 autoimmune hepatitis is the best unifying diagnosis. The decisive features are a markedly hepatocellular liver-enzyme pattern, elevated IgG, and positive ANA and smooth-muscle antibodies. Arthralgia and ANA positivity can lead to an initial rheumatology referral, but neither is specific for connective-tissue disease. SLE is unlikely without characteristic clinical manifestations, anti-dsDNA/ENA antibodies or complement consumption. Primary biliary cholangitis usually produces a predominantly cholestatic pattern with substantially raised ALP and is commonly associated with antimitochondrial antibodies; corresponding PBC features are also required for an overlap syndrome. Drug-induced autoimmune-like hepatitis requires a credible temporal exposure. Confirmation of autoimmune hepatitis requires specialist assessment, exclusion of alternative causes and usually liver biopsy.

Reference: North West London Pathology, Liver autoantibody Screen/Liver Kidney Stomach (LKS) Screen, updated 27 March 2026. https://www.nwlpathology.nhs.uk/test/liver-autoantibody-screen-liver-kidney-stomach-lks-screen/