History
A 62-year-old lady with good past health presents with jaundice and tea-coloured urine. She denies abdominal pain or fever. She is a non-drinker with no history of intravenous drug abuse. She has taken some Chinese herbal drink previously but denies any other over-the-counter medications in the recent few weeks.
•Afebrile, pulse 71 bpm, BP 124/60mmHg, SaO2 98% on RA.
•Hydration is satisfactory.
•Examination of the hands reveals no clubbing and normal-appearing palmar creases.
•Head and neck examination is unremarkable.
•Cardiovascular: HS dual, no murmur.
•Her chest is clear on auscultation.
•Abdominal examination reveals a soft, non-tender abdomen, with no distension.
•GCS 15/15.
•No focal neurology.
•No flapping tremor.
•No signs of oedema.
Investigations
•CBC:
–WBC 5 x 109/L;
–haemoglobin 14g/dL;
–platelets 243 x 109/L.
•Bilirubin 493μmol/L.
•ALP 193 IU/L.
•ALT 1560 IU/L.
•INR 1.38.
•AFP 5μg/L.
•Albumin 29g/L.
•Globulin 30g/L.
What is your differential diagnosis?
•Viral hepatitis.
•Drug-induced liver injury.
•Autoimmune diseases.
•Wilson’s disease (consider genetic disorders in younger individuals).
What other investigations would you perform?
Further blood tests, including hepatitis serology (for hepatitis A, E, B, C), immunoglobulin pattern and autoimmune markers (anti-nuclear antibody, anti-smooth muscle antibody, anti-mitochondrial antibody are useful for elucidating the cause of hepatitis). All the results come back as negative.
Ultrasound shows no focal hepatic lesions.
The traditional Chinese herbal formula is reviewed by the toxicology team but no obvious hepatotoxic agent could be identified.
How would you proceed?
A liver biopsy is warranted in view of diagnostic uncertainty. Histopathological images are shown below (Figures 17.1 and 17.2).
Please describe what you see
A multinucleated hepatocyte (syncytial giant cell) is present.
Please describe what you see
Prominent interface hepatitis. There is presence of portal plasma cell infiltrates, marked interface hepatitis and emperipolesis. Some scattered multinucleated giant hepatocytes are identified.
What is the likely diagnosis?
The likely diagnosis is autoimmune hepatitis (AIH) given the typical histological features.
Clinical pearls
•Autoimmune hepatitis (AIH) is an inflammatory condition likely caused by the exposure to environmental triggers in a genetically susceptible individual. Treatment can prevent cirrhosis and improve survival.