Autoimmune Hepatitis Treatment Guidelines

First-Line Treatment Options

The American Association for the Study of Liver Diseases recommends a standard first-line treatment for autoimmune hepatitis, which is prednisolone (or prednisone) plus azathioprine, achieving remission in 80-90% of patients and significantly improving survival compared to no treatment 1
The initial dose of prednisolone is 30 mg/day, reducing to 10 mg/day over 4 weeks, plus azathioprine 1-2 mg/kg/day (typically 50 mg/day), with azathioprine initiation when bilirubin levels are below 6 mg/dl, ideally two weeks after starting steroid treatment 2, 1
This combination regimen is associated with significantly fewer corticosteroid-related side effects (10% versus 44%) compared to prednisone monotherapy 2
An alternative option is prednisone alone starting at 60 mg daily, then tapered to 40 mg, 30 mg, and maintenance of 20 mg until endpoint, appropriate for patients with cytopenia, pregnancy, thiopurine methyltransferase deficiency, or malignancy 2, 3
Budesonide (9 mg/day) with azathioprine may be considered as an alternative first-line therapy, but should NOT be used in patients with cirrhosis or acute severe AIH due to risk of systemic side effects 4, 1

The treatment goal is complete normalization of liver enzymes (AST, ALT) and IgG levels, with normalization of laboratory indices before termination of treatment reducing the relative risk of relapse after drug withdrawal by 3-fold to 11-fold 5, 1
Serum aminotransferase levels should improve within 2 weeks of starting therapy, and liver biopsy assessment prior to termination of treatment is recommended to ensure full resolution of the disease 3, 5
Treatment should continue for at least 2 years before considering withdrawal, with failure to achieve complete normalization of liver enzymes and IgG levels leading to almost universal relapse after treatment withdrawal 5

For patients with incomplete response, consider long-term low-dose corticosteroid therapy with gradual decrease to 10 mg daily, and long-term azathioprine (2 mg/kg daily) can be used to stabilize liver enzymes in corticosteroid-intolerant individuals 5
Mycophenolate mofetil (MMF) is recommended as the first second-line agent, particularly for azathioprine intolerance, with an initial dose of 1 g daily, increased to maintenance level of 1.5-2 g daily 6, 7
Tacrolimus (starting dose 0.075 mg/kg daily) may be more effective for patients with refractory disease not responding to standard therapy, and cyclosporine (2-5 mg/kg daily) has shown effectiveness in inducing and maintaining remission, particularly in pediatric patients 8, 7

In children, treatment regimens are similar to adults but with dose adjustments, with early use of azathioprine (1-2 mg/kg daily) or 6-mercaptopurine (1.5 mg/kg daily) recommended to minimize steroid effects on growth, and response to treatment is excellent in children, with normalization of liver tests in 75-90% after 6-9 months 2
For acute severe AIH, high-dose intravenous corticosteroids (≥1 mg/kg) should be administered as early as possible, and if no improvement within 7 days, patients should be evaluated for liver transplantation 1, 3

Cosmetic side effects of corticosteroids occur in 80% of patients after 2 years of treatment, and severe side effects typically develop after 18 months of therapy at prednisone doses >10 mg daily 2, 3
TPMT measurement should be considered before azathioprine initiation to exclude homozygote deficiency, especially in patients with pre-existing leucopenia, and liver biopsy may show persistent interface hepatitis in 55% of patients with normal serum enzymes, highlighting the importance of histological assessment before treatment withdrawal 1, 5