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Review
. 2021 Jun;113(3):185-193.
doi: 10.32074/1591-951X-241.

Pathology of autoimmune hepatitis

Claudia Covelli  1 , Diana Sacchi  2 , Samantha Sarcognato  2 , Nora Cazzagon  3 , Federica Grillo  4 , Francesca Baciorri  2 , Daniela Fanni  5 , Matilde Cacciatore  2 , Valeria Maffeis  2 , Maria Guido  2   6
Affiliations
Review

Pathology of autoimmune hepatitis

Claudia Covelli et al. Pathologica. 2021 Jun.

Abstract

Autoimmune hepatitis (AIH) is a relatively rare non-resolving chronic liver disease, which mainly affects women. It is characterized by hypergammaglobulinemia, circulating autoantibodies, interface hepatitis on liver histology and a favourable response to immunosuppression. The putative mechanism for the development of autoimmune hepatitis is thought to be the interaction between genetic predisposition, environmental triggers and failure of the native immune system.

AIH still remains a major diagnostic and therapeutic challenge, mainly because it is a very heterogeneous disease. Prompt and timely diagnosis is crucial since, if left untreated, AIH has a high mortality rate. Histological demonstration of hepatitis is required for the diagnosis of AIH and, therefore, liver biopsy is mandatory in the initial diagnostic work-up, before treatment. In this review, we summarize the histological features of AIH with the main aim of highlighting the most important clinical-pathological hallmarks useful in the routine diagnostic practice.

Keywords: autoimmune hepatitis; histology; interface hepatitis; liver biopsy.

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Conflict of interest statement

Conflict of interest

The Authors declare no conflict of interest.

Figures

Figure 1.
Figure 1.
Severe portal inflammation, mainly composed of lymphocytes, and interface hepatitis. Several necro-inflammatory foci are visible in the adjacent lobular parenchyma (H&E; original magnification 40x).
Figure 2.
Figure 2.
A cluster of plasma cells is visible close to the portal tract (H&E; original magnification 40x).
Figure 3.
Figure 3.
Immunostain with CD38 helps in identifying a cluster of plasma cells. This is a pediatric case with mild portal inflammation within an otherwise typical clinical presentation (original magnification 20x).
Figure 4.
Figure 4.
Ductular reaction (arrows) around an inflamed portal tract (immunostain for CK7; original magnification 20x).
Figure 5.
Figure 5.
Several foci of “spotty” necrosis, giving the appearance of lobular disarray (H&E; original magnification 20x).
Figure 6.
Figure 6.
Centrilobular necro-inflammation in a case of true acute AIH presentation. Portal tracts were completely spared in this case. Toxic damage is the main differential diagnosis (H&E; original magnification 40x).
Figure 7.
Figure 7.
Typical hepatocyte rosette, representing a regenerative phenomenon in a heavily inflamed liver (H&E; original magnification 60x).
Figure 8.
Figure 8.
Emperipolesis (arrow) appears as a lymphocyte within the hepatocyte cytoplasm (H&E; original magnification 60x).
Figure 9.
Figure 9.
Giant cell transformation in autoimmune hepatitis (H&E; original magnification 40x).
See this image and copyright information in PMC

References

    1. European Association for the Study of the Liver. EASL Clinical Practice Guidelines: autoimmune hepatitis. J Hepatol 2015;63:971-1004. https://doi.org/10.1016/j.jhep.2015.06.030. 10.1016/j.jhep.2015.06.030 Erratum in J Hepatol 2015;63:1543-1544. - DOI - PubMed
    1. Assis DN. Immunopathogenesis of autoimmune hepatitis. Clin Liver Dis (Hoboken) 2020;15:129-132. https://doi.org/10.1002/cld.873 10.1002/cld.873 - DOI - PMC - PubMed
    1. Tanaka A. Autoimmune hepatitis: 2019 update. Gut Liver 2020;14:430-438. https://doi.org/10.5009/gnl19261 10.5009/gnl19261 - DOI - PMC - PubMed
    1. Yeoman AD, Al-Chalabi T, Karani JB, et al. . Evaluation of risk factors in the development of hepatocellular carcinoma in autoimmune hepatitis: implications for follow-up and screening. Hepatology 2008;48:863-870. https://doi.org/10.1002/hep.22432 10.1002/hep.22432 - DOI - PubMed
    1. Wong RJ, Gish R, Frederick T, et al. . Development of hepatocellular carcinoma in autoimmune hepatitis patients: a case series. Dig Dis Sci 2011;56:578-585. https://doi.org/10.1007/s10620-010-1444-6 10.1007/s10620-010-1444-6 - DOI - PubMed

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