From the Guidelines
The workup for autoimmune hepatitis (AIH) includes laboratory tests, imaging, and liver biopsy, with first-line therapy consisting of prednisone (30-60 mg/day) alone or combined with azathioprine (50-150 mg/day) as recommended by the EASL clinical practice guidelines 1. The initial laboratory evaluation should include liver function tests (elevated AST, ALT, bilirubin), complete blood count, and autoantibody testing (ANA, anti-smooth muscle antibody, anti-liver kidney microsomal antibody, and immunoglobulin levels, particularly IgG). Some key points to consider in the workup and treatment of AIH include:
- Imaging studies like ultrasound help exclude other causes of liver disease.
- Liver biopsy remains the gold standard for diagnosis, showing interface hepatitis, lymphoplasmacytic infiltration, and fibrosis.
- Treatment aims to suppress inflammation and prevent progression to cirrhosis.
- The combination of prednisone and azathioprine reduces steroid-related side effects.
- Prednisone is typically tapered over weeks to months to a maintenance dose of 5-10 mg/day while azathioprine is continued.
- Treatment response should be monitored with liver enzymes and IgG levels.
- For patients who don't respond to standard therapy, second-line options include mycophenolate mofetil, cyclosporine, tacrolimus, or biologics like rituximab.
- Treatment is typically long-term, often lifelong, with remission rates of 65-80% as noted in the EASL guidelines 1 and other studies 1. Regular monitoring for medication side effects is essential, including bone density scans for steroid users and complete blood counts for those on azathioprine, as recommended by various guidelines and studies 1. Patients should avoid alcohol and hepatotoxic medications, and those with advanced disease may eventually require liver transplantation, highlighting the importance of careful management and monitoring in AIH patients 1.
From the Research
Workup for Autoimmune Hepatitis
The workup for autoimmune hepatitis involves a combination of clinical, laboratory, and histological findings. The diagnosis is based on:
- Elevation of immunoglobulin G/hypergammaglobulinemia 2
- Detection of characteristic autoantibodies 2, 3, 4
- A typical pattern on liver histology, including interface hepatitis and periportal necrosis 2, 3, 4
- Exclusion of other causes of hepatitis 2, 3, 4
- Response to immunosuppressive treatment, which supports the diagnosis of autoimmune hepatitis 2, 3, 4
Diagnostic Criteria
Diagnostic scores, such as the simplified diagnostic score of the International Autoimmune Hepatitis Group, can help in making the diagnosis, with a sensitivity and specificity of around 90% 5. However, limitations exist, particularly in patients with very acute presentations or atypical cases, where a trial of monotherapy with steroids and quick tapering of the steroids may be recommended 5.
Laboratory Tests
Laboratory tests, including serum aminotransferase levels, autoantibodies, and hypergammaglobulinemia, are essential in the diagnosis and monitoring of autoimmune hepatitis 3, 4, 6. Serum parameters, such as alanine aminotransferase (ALT), aspartate aminotransferase, IgG, and gamma-globulin levels, can be used to assess disease activity and response to treatment 6.
Histological Evaluation
Liver biopsy is the gold standard for assessing disease activity and response to treatment in autoimmune hepatitis 6. However, it is not suitable for regular disease monitoring due to its invasive nature. Histologic remission is reliably indicated by normalization of serum parameters, but about half of the patients with normal serum parameters still show residual histologic activity 6.
Treatment
The mainstay of treatment for autoimmune hepatitis consists of: