Ask AI

Library

Updates

Table of contents

Autoimmune hepatitis

Background

Overview

Definition
AIH is a chronic liver disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, and circulating autoantibodies.
1
Pathophysiology
The cause of AIH is primarily unknown, but there is a complex interplay of genetic (HLA haplotype) and environmental factors.
2
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 2024,2023,2015), 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 ...
Show more

Screening and diagnosis

Clinical presentation: as per AASLD 2020 guidelines, suspect the diagnosis of AIH in all patients presenting with acute or chronic liver disease, including patients with asymptomatic liver test abnormalities, ALF, and autoantibody-negative hepatitis.
E
Create free account

More topics in this section

  • Diagnostic criteria

Diagnostic investigations

Immunoglobulin levels: as per EASL 2015 guidelines, recognize that:
elevated IgG, especially in the absence of cirrhosis, is suggestive of AIH
B
elevated IgG in the absence of IgA and IgM elevation is particularly suggestive of AIH
B
normal IgG or gamma-globulin do not preclude the diagnosis of AIH
most patients with normal IgG demonstrate a fall of IgG levels upon treatment.
B
Create free account

More topics in this section

  • Autoantibody titers

  • Cholangiography

  • Screening for drug-induced liver injury

  • Screening for liver cirrhosis

  • Evaluation for concomitant diseases

  • Psychosocial assessment

Diagnostic procedures

Liver biopsy: as per EASL 2015 guidelines, perform liver biopsy in the initial diagnostic work-up of AIH, as it is a prerequisite for the confirmation of diagnosis.
B
Show 2 more
Create free account

Medical management

General principles: as per EASL 2015 guidelines, initiate treatment in all patients with active AIH.
A
Show 4 more
Create free account

More topics in this section

  • Treatment targets

  • Corticosteroids

  • Immunosuppressive agents

  • Biologics

  • Duration of treatment

  • Maintenance therapy

Surgical interventions

Liver transplantation: as per EASL 2024 guidelines, consider offering early liver transplantation instead of corticosteroid therapy in patients with acute severe AIH with severe coagulopathy and hepatic encephalopathy stages III-IV.
B
Show 2 more
Create free account

Specific circumstances

Pediatric patients, diagnosis: as per ESPGHAN 2018 guidelines, diagnose autoimmune liver disease in pediatric patients based on presence of serum autoantibodies, elevated IgG, compatible liver histology, positive family history for autoimmune conditions, and exclusion of other causes of pediatric chronic liver disease.
E
Show 5 more
Create free account

More topics in this section

  • Pediatric patients (treatment)

  • Pregnant patients

  • Patients with overlap syndromes

  • Patients with acute-on-chronic liver failure

  • Patients with concurrent viral hepatitis

Patient education

General counseling: as per AASLD 2020 guidelines, identify potential barriers to long-term medication compliance and address at the start of treatment and monitor thereafter.
E
Create free account

Preventative measures

Routine immunizations
As per AASLD 2020 guidelines:
Provide routine immunizations in all susceptible patients with AIH according to the age-specific guidelines of the CDC.
E
Provide vaccination against HAV and HBV infections in unprotected patients, preferably before immunosuppressive therapy.
E
Create free account

More topics in this section

  • Prevention of osteoporosis

Follow-up and surveillance

Assessment of treatment response: as per AASLD 2020 guidelines, reevaluate the accuracy of the original diagnosis and medication adherence in patients with AIH not responding to first-line therapy.
E
Create free account

More topics in this section

  • Management of incomplete response

  • Monitoring for relapse

  • Management of relapse

  • Surveillance for malignancy