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Review
. 2023 Jun 9;12(3):77-85.
doi: 10.5409/wjcp.v12.i3.77.

Seronegative autoimmune hepatitis in childhood

Ali Islek  1 , Gokhan Tumgor  2
Affiliations
Review

Seronegative autoimmune hepatitis in childhood

Ali Islek et al. World J Clin Pediatr. .

Abstract

Comprehensive guidelines on seropositive autoimmune hepatitis have been published for both adults and children, although these guidelines comprise only limited knowledge about seronegative autoimmune hepatitis. Autoimmune hepatitis presents as an acute or chronic progressive disease and poor outcomes are inevitable if left untreated. The absence of autoantibody positivity, hypergammaglobulinemia and lack of comprehensive algorithms makes seronegative autoimmune hepatitis a mysterious disease. In general, seronegative autoimmune hepatitis often presents with acute hepatitis, and its treatment and prognosis similar to seropositive autoimmune hepatitis. The present review focuses on the known characteristics of seronegative autoimmune hepatitis in childhood, and those of which current knowledge is vague.

Keywords: Aplastic anemia; Autoantibody negative; Lymphocytopenia; Seronegative autoimmune hepatitis.

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Conflict of interest statement

Conflict-of-interest statement: There was no conflict of interest.

Figures

Figure 1
Figure 1
B cell role in the pathogenesis of autoimmune hepatitis. T cells allow B cells to switch to antibody-producing plasma cells. Autoreactive B cells release autoantibody and cytokine/chemokines. B cells continue to contribute to inflammation by presenting antigen to T cells and cause autoantibody-mediated activation of macrophage/dendritic cells, while suppressing the anti-inflammatory effects of Treg and B cells. Treg: Regulatory T cell.
Figure 2
Figure 2
Algorithm for the diagnosis of seronegative autoimmune hepatitis. 1Proposed scoring criteria for the diagnosis of juvenile autoimmune liver disease by ESPGHAN 2018 (10). AIH: Autoimmune hepatitis; ANA: Anti-nuclear antibody; ASMA: Anti-smooth muscle antibody; LKM: liver-kidney microsomal antibody; IgG: Immunoglobulin G; LC-1: Liver cytosol type-1 antibody; SLA: Anti-soluble liver antigen/liver–pancreas antigen; ASGP-R: Asialoglycoprotein receptor.
See this image and copyright information in PMC

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