MBBS SAQ · Gastroenterology / Hepatology
Autoimmune hepatitis — diagnosis and stepwise management
A final-prof / NEET-PG SAQ on autoimmune hepatitis — hepatitic versus cholestatic pattern, Hennes simplified score, and sourced immunosuppression (prednisolone 0.5 to 1 mg/kg plus azathioprine 1 to 2 mg/kg; budesonide only if non-cirrhotic).
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Question
A 30-year-old woman presents with a 4-week history of fatigue, amenorrhoea and jaundice. She has vitiligo and an aunt with autoimmune thyroid disease. LFTs: ALT 680 U/L, AST 590 U/L, ALP 160 U/L, bilirubin 110 micromol/L. Viral hepatitis screen (HBV/HCV) is negative. IgG is 26 g/L (NR 7-16); ANA is positive at 1:640 and anti-smooth muscle antibody is positive; anti-LKM1 is negative. Liver biopsy shows interface hepatitis. Make the diagnosis, justify it, and outline the stepwise management.[10][12][18]
Model answer
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Diagnosis: autoimmune hepatitis, type 1 serology, acute icteric presentation.[12][18]
The picture is hepatitic (ALT/AST-dominant, ALP only mildly raised). EASL 2025 notes that AIH may affect any patient irrespective of age, sex, or ethnicity and spans asymptomatic disease to acute liver failure. Childhood (and exam) serology still splits type 1 (SMA and/or ANA) from type 2 (anti-LKM1); this patient is type 1.[12][18]
The simplified diagnostic criteria (Hennes 2008) score autoantibodies, immunoglobulin G, histology, and exclusion of viral hepatitis. In the validation set a cutoff of 6 or more had 88% sensitivity and 97% specificity; 7 or more had 81% sensitivity and 99% specificity. The authors proposed probable AIH at greater than 6 points and definite AIH at 7 or higher. Early diagnosis matters because immunosuppression is life-saving.[10]
Why not PBC or PSC? Those are cholestatic (ALP-dominant) bile-duct diseases; here transaminases lead. This is a hepatitic disease — AIH.[10][12]
- Induction. A prospective adult cohort used prednisolone 0.5 to 1 mg/kg/day alone or combined with azathioprine 1 to 2 mg/kg/day (mycophenolate was an alternative first-line in that series). In non-cirrhotic AIH, Manns 2010 showed budesonide 3 mg three times daily (or twice daily) plus azathioprine 1 to 2 mg/kg/day induced complete biochemical remission more often than prednisone 40 mg/day tapered to 10 mg/day plus azathioprine, with fewer steroid-specific side effects — cirrhosis was an exclusion, so do not use budesonide if she is cirrhotic.[11][19]
- Maintenance. Treatment usually needs to be maintained for life, as relapses are common after azathioprine cessation. In childhood series, steroids plus azathioprine induce remission in 80%; only some 20% of type 1 patients discontinue therapy successfully.[18][19]
- Watch for acute severe AIH. If INR is 1.5 or above without cirrhosis or encephalopathy, that cohort definition of AS-AIH received high-dose corticosteroids; delay beyond 5 days predicted non-response and the need for transplant.[13]
- If cirrhosis develops: EASL directs surveillance for portal hypertension and hepatocellular carcinoma, and liver transplantation in decompensated cirrhosis.[12]
Common errors
- Mislabelling as viral hepatitis — exclusion of viral hepatitis is a scored domain; do not skip it.[10]
- Treating a cholestatic (PBC/PSC) picture with steroids — the drug is disease-specific.[10][12]
- Using budesonide in cirrhosis — Manns excluded cirrhosis.[11]
- Withdrawing immunosuppression early — relapses are common after azathioprine cessation; most patients stay on treatment long-term.[18][19]
Examiner notes
- Reward cholestatic versus hepatitic as the first fork.[10][12]
- Reproduce the simplified AIH score domains and the 6 / 7 cutoffs with their validation sensitivities.[10]
- Name a sourced induction pair (prednisolone 0.5 to 1 mg/kg plus azathioprine 1 to 2 mg/kg, or Manns budesonide in non-cirrhotic disease) rather than an unsourced 60 mg tablet dose.[11][19]
References6ShowHide
- [10]Hennes EM, Zeniya M, Czaja AJ, et al. Simplified criteria for the diagnosis of autoimmune hepatitis Hepatology, 2008.PMID 18537184
- [11]Manns MP, Woynarowski M, Kreisel W, et al. Budesonide induces remission more effectively than prednisone in a controlled trial of patients with autoimmune hepatitis Gastroenterology, 2010.PMID 20600032
- [12]European Association for the Study of the Liver. EASL Clinical Practice Guidelines on the management of autoimmune hepatitis J Hepatol, 2025.PMID 40348684
- [18]Mieli-Vergani G, Vergani D. Autoimmune hepatitis in children: what is different from adult AIH? Semin Liver Dis, 2009.PMID 19676002
- [19]Dalekos GN, Arvaniti P, Gatselis NK, et al. Long-term results of mycophenolate mofetil vs. azathioprine use in individuals with autoimmune hepatitis JHEP Rep, 2022.PMID 36411768
- [13]Eshraghian A, Taghavi A, Nikeghbalian S. Outcomes of patients with acute severe autoimmune hepatitis: Predictors of non-response to corticosteroids and need for liver transplantation United European Gastroenterol J, 2024.PMID 38733314