From the Guidelines
The recommended workup for suspected autoimmune hepatitis (AIH) includes comprehensive laboratory testing, imaging, and liver biopsy, with treatment typically beginning with prednisone and azathioprine as a steroid-sparing agent, as recommended by the most recent guidelines 1. The initial laboratory evaluation should include:
- Liver function tests (AST, ALT, alkaline phosphatase, bilirubin)
- Complete blood count
- Coagulation studies
- Autoantibody testing (ANA, anti-smooth muscle antibody, anti-liver kidney microsomal antibody, and immunoglobulin levels, particularly IgG) Viral hepatitis serologies, metabolic tests, and imaging studies should be performed to exclude other liver diseases. A liver biopsy is essential for definitive diagnosis, showing interface hepatitis, lymphoplasmacytic infiltration, and other characteristic features. Treatment with prednisone 40-60 mg daily, which is gradually tapered over several weeks based on clinical and biochemical response, and azathioprine (50-150 mg daily) as a steroid-sparing agent, allowing for lower prednisone maintenance doses (typically 5-10 mg daily), is the first line treatment of AIH, as stated in the EASL clinical practice guidelines 1. For patients with incomplete response or intolerance to standard therapy, alternative immunosuppressants like mycophenolate mofetil, cyclosporine, or tacrolimus may be considered, as suggested by various studies 1. Treatment response should be monitored with regular liver function tests, and therapy is typically continued for at least 2-3 years after biochemical remission before considering withdrawal, with long-term maintenance therapy often necessary to prevent relapse, which occurs in approximately 70% of patients after treatment discontinuation. The most recent guidelines from 2024 1 provide the strongest evidence for the management of autoimmune hepatitis, and should be followed in clinical practice.
From the Research
Diagnostic Workup for Autoimmune Hepatitis
The diagnostic workup for autoimmune hepatitis (AIH) involves a combination of clinical, laboratory, and histological findings. Key features include:
- Elevated transaminase and immunoglobulin G levels 2, 3, 4, 5, 6
- Seropositivity for autoantibodies such as anti-nuclear antibody or anti-smooth muscle antibody 2, 3, 4, 6
- Interface hepatitis and periportal necrosis on liver biopsy 2, 3, 4
- Exclusion of other liver diseases with similar presentations 4
Treatment of Autoimmune Hepatitis
Treatment for AIH typically involves immunosuppressive therapy, with the goal of inducing and maintaining long-term remission of liver inflammation. Recommended treatments include:
- Corticosteroids and azathioprine as first-line therapy 3, 4, 6
- Budesonide with azathioprine as an alternative option 4
- Mycophenolate mofetil as a second-line treatment for patients who do not respond to first-line therapy 2, 6
- Calcineurin inhibitors for difficult-to-treat patients 2, 4
- Liver transplantation for patients with end-stage liver disease or fulminant liver failure 2, 3
Monitoring Disease Activity
Disease activity in AIH can be monitored using a combination of serum markers, including:
- Aminotransferases (ALT and AST) 5, 6
- Immunoglobulin G (IgG) levels 5, 6
- Gamma-globulin levels 5
- Liver biopsy to assess histologic activity 5 Note that normalization of serum parameters is not always a reliable marker for complete histologic remission, but can indicate a low risk of fibrosis progression 5