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Autoimmune hepatitis

What's new

The European Association for the Study of the Liver (EASL) has released a guideline update for the management of autoimmune hepatitis. Immunosuppressive therapy is recommended for all patients with active disease, with corticosteroids in combination with azathioprine as the first-line treatment. Before proceeding to subsequent-line therapies, assessment of nonadherence, treatment optimization, and consideration of allopurinol addition are recommended. Mycophenolate mofetil (MMF) is the preferred second-line option, while tacrolimus, infliximab, rituximab, and belimumab are suggested as third-line rescue therapies. Long-term, often lifelong, continuation of immunosuppressive therapy is required for most patients, using azathioprine or MMF as monotherapy or in combination with low-dose corticosteroids. Patients with decompensated cirrhosis, acute severe autoimmune hepatitis, or autoimmune hepatitis-related acute liver failure should be evaluated for liver transplantation. .

Background

Overview

Definition
AIH is a chronic liver disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, and circulating autoantibodies.
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Pathophysiology
The cause of AIH is primarily unknown, but there is a complex interplay of genetic (HLA haplotype) and environmental factors.
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Disease course
The immunological reaction against autologous liver antigens in genetically predisposed individuals results in AIH, which may an asymptomatic, acute, or acute severe (fulminant) presentation. Disease progression may lead to hepatic encephalopathy, cirrhosis, and a need for the liver transplantation.
3
Prognosis and risk of recurrence
All-cause mortality/liver transplant rates for AIH over a period of 10 years is 13%.
4

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of autoimmune hepatitis are prepared by our editorial team based on guidelines from the European Association for the Study of the Liver (EASL 2025,2024,2023), the World Federation for Ultrasound in Medicine and Biology (WFUMB 2024), the American Association for the Study of Liver Diseases (AASLD 2020), the European Society for Paediatric ...
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Screening and diagnosis

Differential diagnosis: as per EASL 2025 guidelines, include various causes of liver diseases in the differential diagnosis of AIH based on the presentation, such as acute hepatitis, chronic hepatitis, or cirrhosis, as well as extrahepatic conditions such as celiac disease and SLE.
A
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  • Diagnostic criteria

Diagnostic investigations

Serologic testing: as per EASL 2025 guidelines, obtain initial screening for ANA, smooth muscle antibodies, anti-LKM1 antibodies, and anti-LC1 antibodies using IDIF testing on triple rodent tissue sections, in parallel with anti-soluble liver antigen/liver pancreas testing by solid phase assays.
B
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  • Magnetic resonance cholangiography

  • Evaluation for liver cirrhosis

  • Evaluation for variant syndromes

  • Evaluation for concomitant diseases

  • Psychosocial assessment

Diagnostic procedures

Liver biopsy
As per EASL 2025 guidelines:
Perform a liver biopsy to establish the diagnosis of AIH.
A
Ensure the histology report includes grading of necroinflammatory activity, staging of fibrosis, and classification of the findings as likely, possible, or unlikely AIH.
B
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Medical management

General principles: as per EASL 2025 guidelines, initiate immunosuppressive treatment in all patients with active disease, including patients with advanced fibrosis and/or compensated cirrhosis.
A
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  • Induction therapy (first-line)

  • Induction therapy (second-line)

  • Induction therapy (third-line)

  • Maintenance therapy

  • Treatment cessation

  • Management of decompensated cirrhosis

  • Management of acute severe AIH

  • Management of treatment-related adverse events

  • Management of relapse

Surgical interventions

Liver transplantation, indications: as per EASL 2025 guidelines, evaluate patients with AIH and decompensated cirrhosis, acute severe AIH, or AIH-related ALF (including acute-on-chronic liver failure) for liver transplantation in reference centers.
B
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  • Liver transplantation (post-transplant care)

Specific circumstances

Pediatric patients, diagnosis: as per EASL 2025 guidelines, consider using the revised 2018 European Society for Pediatric Gastroenterology, Hepatology and Nutrition scoring system for AIH in pediatric patients.
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  • Pediatric patients (treatment)

  • Pregnant patients (pre-pregnancy care)

  • Pregnant patients (medical therapy)

  • Pregnant patients (monitoring)

  • Elderly patients

  • Patients with drug-induced autoimmune-like hepatitis

  • Patients with variant syndromes

  • Patients with acute-on-chronic liver failure

  • Patients with viral hepatitis

  • Patients with metabolic-associated steatotic liver disease

  • Patients with alcohol-related disease

Preventative measures

Routine immunizations
As per EASL 2025 guidelines:
Provide vaccination against HAV and HBV to all susceptible patients with AIH.
B
Provide all other potential vaccinations, including influenza, SARS-CoV-2, and S. pneumoniae, in accordance with national guidelines.
B
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Follow-up and surveillance

Monitoring for treatment adherence: as per EASL 2025 guidelines, provide patient-centered consultations to assess for anxiety, depression, and other reasons for suspected or confirmed nonadherence, including evaluation of capability, opportunity, and motivation.
B
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  • Monitoring for complications

  • Monitoring for relapse

  • Surveillance for malignancy