What are the diagnostic tests and treatment options for autoimmune hepatitis?

From the Guidelines

Diagnosing and treating autoimmune hepatitis requires a comprehensive approach, including blood tests, imaging, liver biopsy, and immunosuppressive therapy, with the goal of achieving and maintaining remission to prevent permanent liver damage. The diagnostic tests for autoimmune hepatitis typically involve:

• Blood tests, including liver function tests (elevated ALT, AST), immunoglobulin levels (particularly elevated IgG), and autoantibody tests (ANA, anti-smooth muscle antibody, anti-liver kidney microsomal antibody) 1
• Imaging studies, such as ultrasound or MRI, to assess liver morphology and rule out other liver diseases
• Liver biopsy, which is often necessary to confirm the diagnosis and assess disease severity 1 Treatment focuses on suppressing the immune system to reduce liver inflammation, with first-line therapy usually combining prednisone (starting at 30-60 mg daily and gradually tapering) with azathioprine (50-150 mg daily), as recommended by the EASL clinical practice guidelines 1. This combination helps reduce steroid side effects while maintaining disease control. For patients who don't respond to standard therapy, alternative immunosuppressants like mycophenolate mofetil, cyclosporine, or tacrolimus may be used. Treatment typically continues long-term, often for years, with the goal of achieving and maintaining remission. Regular monitoring of liver function tests and medication side effects is essential to prevent complications and adjust treatment as needed. Without treatment, autoimmune hepatitis can progress to cirrhosis, liver failure, and may eventually require liver transplantation, which is why prompt diagnosis and treatment are crucial for preventing permanent liver damage 1.

From the Research

Diagnostic Tests for Autoimmune Hepatitis

• The diagnosis of autoimmune hepatitis is based on the presence of autoantibodies, such as antinuclear antibody, smooth muscle antibody, liver-kidney microsomal type 1 antibody, and perinuclear antineutrophilic cytoplasmic antibody 2
• The International Autoimmune Hepatitis Group (IAIHG) has established diagnostic scores, including the revised and simplified diagnostic scores, which are based on clinical, laboratory, and histological parameters 3, 4
• Autoantibody detection is mandatory for the diagnosis and classification of autoimmune hepatitis, with a sensitivity and specificity of around 90% in different populations 3, 4
• Liver biopsy is also an important diagnostic tool, which can reveal severe active hepatitis with mononuclear cell infiltration in portal areas and piecemeal necrosis 2

Treatment Options for Autoimmune Hepatitis

• The treatment of autoimmune hepatitis typically involves the use of immunosuppressive drugs, such as prednisone and azathioprine 2
• The initial treatment consists of prednisone (2 mg/kg/day) per oral, with the addition of azathioprine (1.5 mg/kg/day) per oral at the 3rd-6th month, and slow tapering of prednisone 2
• Azathioprine administration is necessary to decrease the prednisone dose and maintain sustained normal transaminase values 2
• The treatment response is monitored by assessing serum parameters, such as aminotransferases, IgG, and gamma-globulin levels, which are significantly associated with histologic activity 5

Monitoring Disease Activity

• Disease activity and response to treatment in autoimmune hepatitis are assessed best by liver biopsy, but this is not suitable for regular disease monitoring 5
• Serum parameters, such as ALT and IgG, are commonly used to monitor disease activity, with elevated levels indicating high inflammatory activity 5
• Normalization of serum parameters is not a reliable marker for complete histologic remission, but it can identify patients at low risk of fibrosis progression 5