Heterogeneity of Liver Disease in Common Variable Immunodeficiency Disorders.
antibody deficiency
common variable immune deficiency
liver disease
liver transplant
monogenic immune defects
nodular regenerative hyperplasia
primary immuno deficiency
transient elastography
Journal
Frontiers in immunology
ISSN: 1664-3224
Titre abrégé: Front Immunol
Pays: Switzerland
ID NLM: 101560960
Informations de publication
Date de publication:
2020
2020
Historique:
received:
21
10
2019
accepted:
11
02
2020
entrez:
19
3
2020
pubmed:
19
3
2020
medline:
5
3
2021
Statut:
epublish
Résumé
Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency (PID) in adulthood and is characterized by severe reduction of immunoglobulin serum levels and impaired antibody production in response to vaccines and pathogens. Beyond the susceptibility to infections, CVID encompasses a wide spectrum of clinical manifestations related to a complex immune dysregulation that also affects liver. Although about 50% CVID patients present persistently deranged liver function, burden, and nature of liver involvement have not been systematically investigated in most cohort studies published in the last decades. Therefore, the prevalence of liver disease in CVID widely varies depending on the study design and the sampling criteria. This review seeks to summarize the evidence about the most relevant causes of liver involvement in CVID, including nodular regenerative hyperplasia (NRH), infections and malignancies. We also describe the clinical features of liver disease in some monogenic forms of PID included in the clinical spectrum of CVID as ICOS, NFKB1, NFKB2, CTLA-4, PI3Kδ pathway, ADA2, and IL21-R genetic defects. Finally, we discuss the clinical applications of the various diagnostic tools and the possible therapeutic approaches for the management of liver involvement in the context of CVID.
Identifiants
pubmed: 32184784
doi: 10.3389/fimmu.2020.00338
pmc: PMC7059194
doi:
Substances chimiques
CTLA-4 Antigen
0
Inducible T-Cell Co-Stimulator Protein
0
NF-kappa B
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
338Informations de copyright
Copyright © 2020 Pecoraro, Crescenzi, Varricchi, Marone and Spadaro.
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