Autoimmune Hepatitis Treatment Guidelines

Introduction to Treatment

The American Association for the Study of Liver Diseases recommends initiating treatment with prednisone and azathioprine immediately in patients with moderate-to-severe autoimmune hepatitis, achieving biochemical remission in 80% of patients within 6 months 1, 2.

The preferred treatment regimen is combination therapy with prednisone plus azathioprine, with a standard dosing regimen of prednisone 30 mg/day + azathioprine 50 mg/day (US) or 1-2 mg/kg/day (Europe) for week 1, tapering to maintenance doses 1, 2, 5.
For patients with severe hyperbilirubinemia, start prednisone first, then add azathioprine after 2 weeks as a safer approach 3, 5.

Budesonide 9 mg/day plus azathioprine may be used in treatment-naive, non-cirrhotic patients with early-stage disease who face high risk of steroid side effects, but should not be used in patients with cirrhosis or acute severe AIH 3, 5, 6.

Continue treatment for at least 2 years and for at least 12 months after normalization of liver enzymes, with complete normalization of BOTH serum aminotransferases AND IgG levels as the treatment goal 3, 5.

Serum aminotransferases should improve within 2 weeks of starting therapy, and assess response at 4-8 weeks after treatment initiation 3, 4.

For confirmed non-responders after 3 years, increase to high-dose therapy: prednisone 60 mg daily alone OR prednisone 30 mg daily plus azathioprine 150 mg daily (or up to 2 mg/kg/day) for at least 1 month 3, 5.
For steroid-refractory cases, use alternative second-line agents including tacrolimus, cyclosporine, or mycophenolate mofetil, with tacrolimus superior to MMF at achieving biochemical remission in patients who had not responded to standard therapy 3, 6.

Treat acute severe AIH immediately with high-dose intravenous corticosteroids (≥1 mg/kg) as early as possible, and list for emergency liver transplantation if no improvement occurs within 7 days 3, 4.

All patients must receive calcium and vitamin D supplementation from treatment initiation, and monitor bone mineral density with DEXA scanning at 1-2 year intervals 1, 2, 3.

Relapse occurs in 50-90% of patients within 12 months of stopping treatment, and consider long-term maintenance with azathioprine 2 mg/kg/day, which maintains remission in 83-87% of adult patients during median follow-up of 67 months 3, 5.

For children, prednisone is the mainstay at 1-2 mg/kg daily (up to 60 mg/day) for two weeks, then taper over 6-8 weeks to 0.1-0.2 mg/kg daily or 5 mg daily with azathioprine, with early use of azathioprine recommended for all children without contraindications 1, 2.