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Autoimmune hepatitis

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Updated 2019 HASL and 2018 ESPGHAN guidelines for the diagnosis and management of 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.
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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 2023,2015), the American Association for the Study of Liver Diseases (AASLD 2020), the Hellenic Association for the Study of the Liver (HASL 2019), the European Society for Paediatric...
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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.
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  • Diagnostic criteria

Classification and risk stratification

Classification
As per HASL 2019 guidelines:
Classify the diagnosis of acute AIH as one of the following two clinical forms of presentation:
acute worsening of previously undiagnosed or misdiagnosed AIH
real (original) acute onset of AIH without chronic lesions on liver histology
B
Consider classifying AIH into two types:
AIH-1: ANA, smooth muscle antibodies and/or anti-SLA/LP positive
AIH-2: anti-LKM1, anti-LKM3 and/or anti-LC1 positive
C
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Diagnostic investigations

Immunoglobulin levels: as per HASL 2019 guidelines, Do not exclude the diagnosis of AIH in patients with normal IgG levels, even though a selective high serum IgG is an important AIH characteristic.
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  • 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
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Medical management

General principles: as per HASL 2019 guidelines, Treat all patients with active disease, even with advanced fibrosis or cirrhosis.
A
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  • Treatment targets

  • Corticosteroids

  • Immunosuppressive agents

  • Biologics

  • Duration of treatment

  • Maintenance therapy

Surgical interventions

Liver transplantation: as per AASLD 2020 guidelines, Evaluate patients with AIH for liver transplantation in the following circumstances:
ALF
failed to improve laboratory tests within 1-2 weeks of corticosteroid therapy
clinical deterioration within 1-2 weeks of corticosteroid therapy.
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Specific circumstances

Pediatric patients, diagnosis: as per HASL 2019 guidelines, Obtain at least MRCP in pediatric patients with AIH to exclude autoimmune sclerosing cholangitis.
B
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  • Pediatric patients (treatment)

  • Pregnant patients

  • Elderly patients

  • Patients with overlap syndromes

  • Patients with acute-on-chronic liver failure

  • Patients with fatty liver disease

  • Patients with concurrent viral hepatitis

  • Patients with HIV co-infection

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.
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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.
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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.
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  • Management of incomplete response

  • Monitoring for relapse

  • Management of relapse

  • Surveillance for malignancy