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Autoimmune hepatitis
Autoimmune hepatitis is chronic liver inflammation with autoantibodies (ANA, smooth muscle antibodies) and a high IgG, confirmed by biopsy and responding well to steroids with azathioprine; untreated, it progresses to cirrhosis.
Why it matters in the exam
The typical question presents a young or middle-aged woman with fatigue, jaundice or just raised transaminases, sometimes with another autoimmune disease (thyroid, type 1 diabetes, coeliac). The candidate must choose the work-up: ANA, smooth muscle antibodies (ASMA), IgG — and in children anti-LKM1 antibodies (type 2) — and exclusion of other causes: viruses, alcohol, Wilson disease and drugs that mimic the disease (nitrofurantoin, minocycline). The diagnosis is confirmed by biopsy: interface hepatitis with plasma cells. Remember that about one in ten patients presents without the usual antibodies and with a normal IgG, so a biopsy is needed even when serology is negative but clinical suspicion is high; the biopsy also sets the fibrosis stage, which may already be advanced at diagnosis.
Treatment: prednisone (or budesonide when there is no cirrhosis) together with azathioprine, after a TPMT test. The goal is a normal ALT and IgG, and withdrawal is considered only after at least two years of remission — and relapse is common. Failure or intolerance — mycophenolate. Severe acute autoimmune hepatitis with a high INR needs early referral to a transplant centre. A well-known distractor: "watch and wait" with high transaminases, a high IgG and antibodies — every delay adds fibrosis. Overlap with PBC or PSC is possible, so with marked cholestasis AMA and MRCP are also checked.
Key points
- Woman, raised transaminases, ANA and smooth muscle antibodies, high IgG.
- Biopsy confirmation: interface hepatitis with plasma cells.
- Prednisone or budesonide + azathioprine (after TPMT).
- Goal normal ALT and IgG; two years of remission before trying withdrawal.
- Exclude drugs, viruses and Wilson disease.
Summary table
| Topic | Finding | Action | Exam note |
|---|---|---|---|
| Type 1 | ANA, smooth muscle antibodies, high IgG | Biopsy | Commonest in adults |
| Type 2 | Anti-LKM1 | Biopsy | Mainly children |
| Exclusion | Viruses, alcohol, Wilson, drugs (nitrofurantoin, minocycline) | Serology and ceruloplasmin | Drug-induced hepatitis mimics the disease |
| Biopsy | Interface hepatitis with plasma cells | Confirms the diagnosis | Also stages fibrosis |
| Treatment | Prednisone or budesonide + azathioprine | Goal: normal ALT and IgG | TPMT before azathioprine |
| Maintenance | At least two years of remission before trying withdrawal | Mycophenolate on failure | Relapse is common |
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Sources
- AASLD Practice Guidance and Guidelines: Diagnosis and Management of Autoimmune Hepatitis in Adults and Children, 2019 — Serology, biopsy and immunosuppressive therapy
- Harrison's Principles of Internal Medicine, 21st edition (2022) — Chronic Hepatitis: Autoimmune Hepatitis
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.