From the Guidelines
Autoimmune hepatitis is diagnosed through a combination of clinical features, laboratory tests, and liver biopsy, and treatment aims to suppress inflammation and prevent progression to cirrhosis, with first-line therapy consisting of prednisone and azathioprine, as recommended by the EASL clinical practice guidelines 1. The diagnosis of autoimmune hepatitis typically requires elevated liver enzymes (ALT, AST), increased serum IgG levels, and positive autoantibodies such as ANA, anti-smooth muscle antibody (ASMA), or anti-liver kidney microsomal antibody (anti-LKM1) 1.
- Key laboratory tests include:
- Liver enzymes (ALT, AST)
- Serum IgG levels
- Autoantibodies (ANA, ASMA, anti-LKM1)
- A liver biopsy showing interface hepatitis with lymphoplasmacytic infiltration is crucial for confirming the diagnosis, with typical features including interface hepatitis, lymphocytic/lymphoplasmacytic infiltrates, emperipolesis, and hepatic rosette formation 1. Treatment response should be monitored with liver enzymes and IgG levels, with the goal of complete biochemical normalization, as recommended by the EASL clinical practice guidelines 1.
- The treatment approach includes:
- First-line therapy: prednisone and azathioprine
- Monitoring: liver enzymes, IgG levels, and complete blood counts
- Duration: at least 2-3 years before considering withdrawal in patients with sustained remission For those who don't respond to standard therapy, second-line options include mycophenolate mofetil, tacrolimus, or cyclosporine, and regular monitoring for medication side effects is essential, including bone density scans for patients on long-term steroids and complete blood counts for those on azathioprine 1. Without treatment, autoimmune hepatitis can progress to cirrhosis, liver failure, and may eventually require liver transplantation, highlighting the importance of prompt diagnosis and treatment, as recommended by the EASL clinical practice guidelines 1.
From the Research
Diagnostic Approaches for Autoimmune Hepatitis
- Autoimmune hepatitis is characterized by increased serum aminotransferase levels, autoantibodies, hypergammaglobulinemia, and interface hepatitis 2
- Diagnosis requires multiple findings and exclusion of similar diseases, with histological analysis remaining a cornerstone of diagnosis 3
- Diagnostic scoring systems support clinical judgment and serve as valuable tools in diagnosis and research 3
- A specific set of autoantibodies, increased IgG concentrations, and histological demonstration of interface hepatitis and periportal necrosis are the diagnostic hallmarks of autoimmune hepatitis 4
Laboratory Tests for Autoimmune Hepatitis
- Liver-related autoantibodies are crucial for the correct diagnosis and classification of autoimmune liver diseases, including autoimmune hepatitis types 1 and 2 (AIH-1 and 2) 5
- AIH-1 is specified by anti-nuclear antibody (ANA) and smooth muscle antibody (SMA), while AIH-2 is specified by antibody to liver kidney microsomal antigen type-1 (anti-LKM1) and anti-liver cytosol type 1 (anti-LC1) 5
- The presence of anti-smooth muscle autoantibody (SMA) in Autoimmune Hepatitis (AIH) is well established, but its predictive value for AIH in patients with normal liver function is low 6
Treatment Approaches for Autoimmune Hepatitis
- Treatment with prednisone or prednisolone with azathioprine is recommended, with budesonide with azathioprine being an alternative option with fewer side effects 2
- Calcineurin inhibitors and mycophenolate mofetil are salvage agents in small series, and liver transplantation is effective for liver failure 2
- The aims of treatment are to induce and maintain long-term remission of liver inflammation, with most patients requiring lifelong maintenance therapy 4