Management of Autoimmune Hepatitis
The standard first-line treatment for autoimmune hepatitis is a combination of prednisolone and azathioprine, which achieves remission in over 80% of patients and should be initiated promptly to prevent progression to cirrhosis and liver failure. 1
First-Line Treatment
Initial Therapy
- Prednisolone: Start at 30-60 mg/day (0.5-1 mg/kg/day)
- Azathioprine: Start at 50 mg/day, increasing to 1-2 mg/kg/day maintenance dose
Recommended Tapering Schedule (for a 60 kg patient)
| Week | Prednisolone (mg/day) | Azathioprine (mg/day) |
|---|---|---|
| 1 | 60 | - |
| 2 | 50 | - |
| 3 | 40 | 50 |
| 4 | 30 | 50 |
| 5 | 25 | 100 |
| 6 | 20 | 100 |
| 7-8 | 15 | 100 |
| 9-10 | 12.5 | 100 |
| >10 | 10 | 100 |
Alternative First-Line Option
- Budesonide (9 mg/day) + azathioprine for patients without cirrhosis, severe acute hepatitis, or acute liver failure 1
- Budesonide has 90% first-pass hepatic clearance and is contraindicated in cirrhotic patients or those with portosystemic shunts
Monitoring and Treatment Duration
- Weekly liver tests and blood counts for first 4 weeks, then monthly once stable
- Clinical improvement should occur within 2 weeks
- 80-90% of patients achieve laboratory remission within 6-12 months
- Minimum treatment duration: 24 months 1
- Consider liver biopsy after 2 years to confirm histological remission
- Lifelong clinical and biochemical monitoring is mandatory, even after treatment cessation 2
Treatment Goals
- Complete biochemical remission (normalization of serum aminotransferases and IgG levels)
- Histological resolution of inflammation
- Prevention of disease progression, cirrhosis, and liver-related mortality
Management of Treatment Failure or Intolerance
For Azathioprine Intolerance
- Mycophenolate mofetil (MMF): First choice for azathioprine intolerance (58% response rate) 2, 1
- Start at 1 g twice daily
For Refractory Disease (Non-responders)
- Increase azathioprine dose to 2 mg/kg/day if failing after 2 years on standard therapy 2
- Tacrolimus: More effective than MMF for non-responders (56% vs 34% remission rate) 1
- Cyclosporine: 2-5 mg/kg daily
- Target trough levels: 100-300 ng/mL 2
Special Considerations
Prednisolone Monotherapy (60 mg/day initially)
Appropriate for:
- Patients with cytopenia who cannot tolerate azathioprine
- Pregnant patients
- Patients with thiopurine methyltransferase (TPMT) deficiency
Prophylaxis and Monitoring for Side Effects
- Calcium and vitamin D supplementation for all patients on steroids
- DEXA scanning at 1-2 year intervals
- Test for TPMT activity prior to azathioprine initiation
- Vaccination against hepatitis A and B should be performed early in susceptible patients 2
Relapse Management
- 50-90% of patients relapse within 12 months of stopping treatment 2
- Reintroduce initial treatment regimen (prednisolone + azathioprine)
- Over 80% achieve biochemical remission again, usually within a few months
Liver Transplantation
Consider referral for transplantation in:
- Patients with decompensation at presentation
- Severe disease with no or slow response to treatment
- Fulminant hepatic failure
- Clinical liver decompensation
- High MELD or Child-Pugh scores
Factors Associated with Poor Outcomes
- Type 2 AIH and SLA positive AIH
- Cirrhosis at presentation
- Confluent necrosis on biopsy
- Persistent AST elevation
- Failure to achieve remission over 2 years
- Multiple relapses 2
Emerging Therapies
For difficult-to-treat cases, infliximab has shown promise in small studies but may be associated with infectious complications 4