From the Guidelines
Diagnosing and treating autoimmune hepatitis requires a comprehensive approach, including blood tests, imaging, and liver biopsy, with treatment typically starting with prednisolone and azathioprine as the first line of therapy, as recommended by the EASL clinical practice guidelines 1. The diagnostic process involves a combination of tests, including:
- Liver function tests to detect elevated liver enzymes (ALT, AST)
- Immunoglobulin levels, particularly elevated IgG
- Autoantibody screening for ANA, anti-smooth muscle antibodies (ASMA), and anti-liver kidney microsomal antibodies (anti-LKM1) A liver biopsy is essential for definitive diagnosis, showing characteristic interface hepatitis and plasma cell infiltration. The simplified diagnostic criteria for AIH, as outlined in the EASL guidelines 1, include:
- Increased serum c-globulin or IgG levels
- Presence of autoantibodies, such as ANA, SMA, or LKM
- Compatible or typical liver histology
- Absence of viral hepatitis Treatment should aim to normalize liver enzymes and reduce inflammation, with the goal of achieving full biochemical remission, as defined by normal levels of transaminases and IgG 1. The treatment typically begins with prednisolone, followed by the addition of azathioprine after two weeks, as the first line of therapy 1. For patients who don't respond to standard therapy, second-line options may be considered, including mycophenolate mofetil, cyclosporine, tacrolimus, or biologics like rituximab. Regular monitoring of liver function tests, complete blood counts, and medication side effects is crucial, as well as monitoring for complications of both the disease and its treatment, including osteoporosis, diabetes, and increased infection risk.
From the Research
Diagnostic Tests for Autoimmune Hepatitis
- Autoimmune hepatitis (AIH) is characterized by interface hepatitis with lymphoplasmacellular infiltrates on liver biopsy, high serum globulin level, and circulating autoantibodies 2
- The diagnosis of AIH is based on a scoring system codified by an international consensus, which includes clinical presentation, serology, and histology 2, 3
- Autoantibody profile is used to classify AIH into two types: type 1 is characterized by anti-nuclear (ANA) and/or anti-smooth muscle (SMA) antibodies, and type 2 by anti-liver kidney microsomal type 1 (anti-LKM-1) antibodies 2
- Histological analysis remains the cornerstone of diagnosis, and biopsy is essential to make the diagnosis 3
Treatment Options for Autoimmune Hepatitis
- Corticosteroids alone or in conjunction with azathioprine is the treatment of choice in patients with AIH, resulting in remission induction in over 80% of patients 2
- Alternative proposed strategies in patients who have failed to achieve remission on standard therapy or patients with drug toxicity include the use of cyclosporine, tacrolimus, budesonide, or mycophenolate mofetil 2, 4
- Liver transplantation is the treatment of choice in managing decompensated disease, although AIH can recur or develop de novo after liver transplantation 2
- Treatment with prednisone or prednisolone with azathioprine is recommended, and budesonide with azathioprine has normalized laboratory tests with few side effects 4
- The aims of treatment are to induce and maintain long-term remission of liver inflammation, and most patients need lifelong maintenance therapy 5
Noninvasive Biomarkers for Autoimmune Hepatitis
- Biomarkers that can predict the clinical course of disease, identify patients at elevated risk for relapse, and improve monitoring and medication dosing beyond current practice would have high clinical value 6
- Novel candidate biomarkers in adult and pediatric AIH include gene expression profiles, proteins, metabolites, and immune cell phenotypes in different stages of AIH 6