What is the typical treatment for autoimmune hepatitis?

Treatment of Autoimmune Hepatitis

The standard first-line treatment for autoimmune hepatitis is prednisolone (or prednisone) plus azathioprine, which achieves remission in 80-90% of patients and significantly improves survival compared to no treatment. 1

First-Line Treatment Options

Standard Combination Therapy

• Prednisolone 30 mg/day initially, reducing to 10 mg/day over 4 weeks, plus azathioprine 1-2 mg/kg/day (typically 50 mg/day) 2, 1
• Azathioprine should be initiated when bilirubin levels are below 6 mg/dl, ideally two weeks after starting steroid treatment 1
• This combination regimen is associated with significantly fewer corticosteroid-related side effects (10% versus 44%) compared to prednisone monotherapy 2

Prednisone Monotherapy

• Alternative option: prednisone alone starting at 60 mg daily, then tapered to 40 mg, 30 mg, and maintenance of 20 mg until endpoint 2, 3
• Appropriate for patients with cytopenia, pregnancy, thiopurine methyltransferase deficiency, or malignancy 2

Budesonide Option

• Budesonide (9 mg/day) with azathioprine may be considered as an alternative first-line therapy 2
• Should NOT be used in patients with cirrhosis or acute severe AIH due to risk of systemic side effects 1

Treatment Goals and Monitoring

• The treatment goal is complete normalization of liver enzymes (AST, ALT) and IgG levels 2, 1
• Normalization of laboratory indices before termination of treatment reduces the relative risk of relapse after drug withdrawal by 3-fold to 11-fold 2
• Serum aminotransferase levels should improve within 2 weeks of starting therapy 3
• Liver biopsy assessment prior to termination of treatment is recommended to ensure full resolution of the disease 2
• Treatment should continue for at least 2 years before considering withdrawal 2

Management of Treatment Failure or Intolerance

Incomplete Response

• For patients with incomplete response (improved but not normalized laboratory values after 36 months), consider long-term low-dose corticosteroid therapy with gradual decrease to 10 mg daily 2
• Long-term azathioprine (2 mg/kg daily) can be used to stabilize liver enzymes in corticosteroid-intolerant individuals 2

Second-Line Options

• Mycophenolate mofetil (MMF) is recommended as the first second-line agent, particularly for azathioprine intolerance (effective in 58% of such cases) 2
• Initial dose of MMF typically 1 g daily, increased to maintenance level of 1.5-2 g daily 2
• Tacrolimus (starting dose 0.075 mg/kg daily) may be more effective for patients with refractory disease not responding to standard therapy 2
• Cyclosporine (2-5 mg/kg daily) has shown effectiveness in inducing and maintaining remission, particularly in pediatric patients 2, 4

Special Populations

Children

• Treatment regimens similar to adults but with dose adjustments: prednisone 1-2 mg/kg daily (up to 60 mg daily) initially 2
• Early use of azathioprine (1-2 mg/kg daily) or 6-mercaptopurine (1.5 mg/kg daily) is recommended to minimize steroid effects on growth 2
• Response to treatment is excellent in children, with normalization of liver tests in 75-90% after 6-9 months 2

Acute Severe AIH

• High-dose intravenous corticosteroids (≥1 mg/kg) should be administered as early as possible 1, 3
• If no improvement within 7 days, patients should be evaluated for liver transplantation 3

Common Pitfalls and Considerations

• Failure to achieve complete normalization of liver enzymes and IgG levels leads to almost universal relapse after treatment withdrawal 2
• Cosmetic side effects of corticosteroids (facial rounding, dorsal hump, striae, weight gain) occur in 80% of patients after 2 years of treatment 2
• Severe side effects (osteoporosis, diabetes, psychosis, hypertension) typically develop after 18 months of therapy at prednisone doses >10 mg daily 3
• TPMT measurement should be considered before azathioprine initiation to exclude homozygote deficiency, especially in patients with pre-existing leucopenia 1
• Liver biopsy may show persistent interface hepatitis in 55% of patients with normal serum enzymes, highlighting the importance of histological assessment before treatment withdrawal 2