Autoimmune Hepatitis Management
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Direct answer
Raised aminotransferases with a high globulin fraction in a young woman — plus interface hepatitis with plasma cells on biopsy and characteristic autoantibodies — define autoimmune hepatitis (AIH), type 1 (ANA and anti-smooth muscle antibody, any age) and type 2 (anti-LKM-1, girls and young women, more aggressive course). Diagnosis is scored, not guessed: the simplified IAIHG score awards points for autoantibody titre, raised IgG, compatible histology and absence of viral hepatitis, with six or more points making probable and seven definite AIH. Treatment is induction with prednisolone 0.5-1 mg/kg/day (or budesonide in non-cirrhotics) with azathioprine 50 mg/day introduced alongside and titrated to 1-2 mg/kg/day for maintenance; withdrawal is considered only after complete biochemical normalisation and, ideally, histological remission, because relapse off therapy is near-universal after a first withdrawal.
What you must remember
- The autoantibody map: type 1 — ANA and anti-SMA; type 2 — anti-LKM-1 (younger, severe, can be anti-LKM-1 alone with normal IgG); anti-soluble liver antigen (anti-SLA/LP) is the most specific antibody across both types and predicts relapse.
- Simplified score arithmetic: autoantibody titre (1 point for 1:40, 2 for 1:80 or more), IgG above 1.1 times upper limit (1 point) or 1.3 times (2 points), histology compatible (1) or typical interface hepatitis with plasma cells (2), absence of viral hepatitis (2) — six probable, seven definite.
- Induction regimen to recite: prednisolone 0.5-1 mg/kg/day tapering over weeks to a maintenance of 5-10 mg, with azathioprine 1-2 mg/kg/day as the steroid-sparing partner; budesonide 9 mg daily is an alternative induction in non-cirrhotic patients to spare bone and metabolism.
- Biochemical endpoints: normalisation of transaminases and IgG — a persistently raised IgG during "remission" predicts histological activity and relapse; repeat biopsy after 1-2 years of remission guides withdrawal decisions.
- The acute severe presentation: jaundice and coagulopathy with AIH serology — biopsy before immunosuppression where safe, steroids in a monitored setting, and a low threshold for transplant listing because acute severe AIH can fail medical therapy within days.
- Overlap syndromes: AIH with primary biliary cholangitis (AMA positive, cholestatic enzymes) or with primary sclerosing cholangitis (duct changes on cholangiography) — treated with AIH therapy plus UDCA 13-15 mg/kg/day in the PBC overlap; paediatric and inflammatory bowel disease cohorts have the most PSC overlap.
- Azathioprine safety file: check TPMT activity before full dosing; watch for leucopenia and pancreatitis; avoid in pregnancy at conception? — no, azathioprine is continued in pregnancy; avoid starting it de novo during pregnancy.
- Drug-induced AIH mimic: nitrofurantoin, minocycline, statins, infliximab and checkpoint inhibitors can reproduce the serology and histology — a drug history precedes lifelong immunosuppression.
Diagnosis by score
A 26-year-old woman presents with six weeks of fatigue, jaundice, AST 640, ALT 720, bilirubin 6, IgG 2,200 mg/dL (1.6 times the upper limit), ANA 1:320, anti-SMA positive, hepatitis B and C serology negative. Scoring runs: autoantibodies 2, IgG 2, and the biopsy — interface hepatitis with plasma cell infiltrates and emperipolesis — earns the full 2, absence of viral hepatitis 2; total 8, definite AIH. Prednisolone 60 mg daily begins, tapering on a scheduled slide; azathioprine 50 mg daily starts at week two once TPMT is confirmed normal, titrating to 100 mg. Transaminases normalise by week ten and IgG by month five; after two years of full remission a withdrawal is discussed but not forced — relapse after first withdrawal exceeds 80 percent, so maintenance at low-dose prednisolone 5 mg with azathioprine is the pragmatic plan, with bone density protection throughout.
The contrast is the icteric 55-year-old with INR 1.8 at presentation: acute severe AIH, admitted, biopsied, transplanted-listed early while steroids are trialled — because non-response in the first week of acute severe disease moves the answer from immunosuppression to transplantation.
Exam and real-world contrasts
Papers exploit the serology map — anti-LKM-1 for type 2, anti-SLA for specificity — and the age-sex script of a young woman with high globulins; the wrong options drag in AMA (that is PBC) and p-ANCA (that is PSC overlap). The second favourite is treatment sequencing: steroid for induction, azathioprine for maintenance — never azathioprine alone for induction, its onset is too slow. Third, the IgG point: candidates forget that IgG is both a diagnostic criterion and a follow-up biomarker; a normal ALT with a raised IgG is not remission. Real-world Indian practice adds the tuberculosis-sieve: before committing to long-term immunosuppression, screen for latent tuberculosis — a chest radiograph, tuberculin or interferon-gamma assay — because steroid-driven reactivation is a feared and entirely preventable complication in this country.
Frequently asked questions
Which antibodies define type 1 and type 2 autoimmune hepatitis?
Type 1 shows ANA and anti-smooth muscle antibodies; type 2 shows anti-liver-kidney microsomal type 1 antibodies, typically in younger patients — anti-soluble liver antigen is the most specific marker across both.
What is the standard induction therapy for AIH?
Prednisolone 0.5-1 mg/kg/day with early introduction of azathioprine 1-2 mg/kg/day for steroid sparing; budesonide 9 mg daily is an alternative induction in non-cirrhotic patients.
How is remission defined and monitored in AIH?
By normalisation of transaminases and IgG — a persistently raised IgG signals ongoing histological activity and predicts relapse, with repeat biopsy guiding any withdrawal attempt.
What is an AIH-PBC overlap syndrome?
Coexistence of AIH serology and histology with positive antimitochondrial antibodies and cholestatic enzymes — treated with corticosteroids plus ursodeoxycholic acid 13-15 mg/kg/day.
Can autoimmune hepatitis present as acute liver failure?
Yes — acute severe AIH with jaundice and coagulopathy warrants urgent biopsy, monitored steroid therapy and early transplant assessment, since medical non-response progresses within days.