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The biological basis of autoimmune liver diseases remains unclear, despite many suggestions.1–3 Not infrequently patients present in ways that demonstrate the artificial boundaries created by clinicians are simply that: classifications inadequately addressing underlying aetiopathogenesis.4
We describe a case that contributes further to our biological understanding. A 33-year-old man was initially diagnosed with primary sclerosing cholangitis (PSC) in 2003 at the time of an asymptomatic and anicteric cholestatic hepatitis: bilirubin 5 μmol/l (normal, <22), alanine aminotransferase (ALT) 221 U/l (normal, <40), aspartate transaminase (AST) 99 U/l (normal, <35), alkaline phosphatase (ALP) 487 U/l (normal, <110), and gamma-glutamyl transferase (GGT) 986 U/l (normal, <45). No other risk factors for liver disease were present either on history, examination or investigation, and pertinently the only immunological abnormality …
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Competing interests: None.
Patient consent: Obtained.