Highlights
- •There is not a single specific laboratory marker to diagnose or exclude AIH.
- •Therefore, diagnosis of AIH is in most cases challenging for physicians.
- •Antibodies detection is mandatory even though not pathognomonic for AIH diagnosis.
- •Assessment of autoimmune serology in strict adherence to guidelines is essential.
- •IgG increase and autoimmunity background support an in-depth serological assessment.
Abstract
Diagnosis of autoimmune hepatitis (AIH) is in most cases challenging for clinicians
as there is not a single specific laboratory or histological marker to diagnose or
exclude the presence of the disease. The clinical spectrum of AIH varies from completely
asymptomatic to acute-severe or even rarely fulminant hepatic failure, while everybody
can be affected irrespective of age, gender, and ethnicity. The old revised and the
newer simplified diagnostic scores have been established by the International Autoimmune
Hepatitis Group (IAIHG) in 1999 and 2008, respectively, which are based on several
clinical, laboratory and histological parameters. Additionally, a thorough differential
diagnosis from other diseases mimicking AIH is absolutely indicated. In this context,
autoantibodies detection in patients with suspected AIH is mandatory -even though
not pathognomonic- not only for AIH diagnosis but furthermore, for AIH classification
(AIH-type 1 and AIH-type 2). Although autoimmune serology can be supportive of AIH
diagnosis in ≥95% of cases if testing has been performed according to the IAIHG guidelines,
this is not the case under real-life circumstances in routine clinical laboratories.
Clinicians should be careful both for the importance of the required testing and how
to interpret the results and therefore, they should communicate and discuss with the
laboratory personnel to achieve the maximum benefit for the patient. Herein, a detailed
and updated review of the diagnostic work-up for AIH diagnosis under real-life conditions
is given to minimize the underestimation and misdiagnosis of AIH which can result
in progression of the disease and unfavourable outcomes.
Keywords
Abbreviations:
AIH (autoimmune hepatitis), IAIHG (International AIH Group), IgG (immunoglobulin G), AIH-1 (autoimmune hepatitis type 1), AIH-2 (autoimmune hepatitis type 2), ANA (antinuclear antibodies), SMA (smooth muscle antibodies), anti-LKM1 (liver kidney microsomal type-1 antibodies), anti-LKM3 (liver kidney microsomal type-3 antibodies), anti-LC1 (liver cytosol type-1 antibodies), anti-SLA/LP (antibodies against soluble liver antigens/liver pancreas), IIF (indirect immunofluorescence), HEp-2 (human larynx epithelioma cancer cell lines), dsDNA (double-stranded DNA), DILI (drug induced liver injury), ELISA (enzyme-linked immunosorbent assay), V-pattern (arterial vessels), VG-pattern (arterial vessels and mesangium of renal glomeruli), VGT-pattern (arterial vessels, glomeruli and intracellular fibrils in renal tubules), F-actin (filamentous actin), anti-α-actinin (antibodies against alpha-actinin), anti-Ro52 (antibodies against ribonucleoprotein/Sjogren's syndrome 52kDa antigen), ANCA (anti-neutrophil cytoplasmic antibodies), cANCA (cytoplasmic ANCA), pANCA (perinuclear ANCA), pANNA (perinuclear antineutrophil nuclear antibody), PSC (primary sclerosing cholangitis), PBC (primary biliary cholangitis), anti-LKM2 (liver kidney microsomal type-2 antibodies), AMA (anti-mitochondrial antibodies), SLE (systemic lupus erythematosus), pIgG (polyreactive IgG)To read this article in full you will need to make a payment
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Article Info
Publication History
Published online: November 16, 2022
Accepted:
November 6,
2022
Received in revised form:
November 4,
2022
Received:
August 20,
2022
Publication stage
In Press Corrected ProofIdentification
Copyright
© 2022 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
