Pediatric autoimmune disorders with gastrointestinal expressions: from bench to bedside.
autoantibody
autoimmune disease
autoimmune enteropathy (AIE)
large bowel
pediatric disease
small bowel
Journal
Pathologica
ISSN: 1591-951X
Titre abrégé: Pathologica
Pays: Italy
ID NLM: 0401123
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
received:
30
07
2021
accepted:
30
07
2021
pubmed:
3
12
2021
medline:
3
3
2022
entrez:
2
12
2021
Statut:
ppublish
Résumé
The gastrointestinal (GI) tract may be involved in systemic autoimmune diseases or may be the target of organ-specific autoimmunity. Autoimmune enteropathy (AIE) is a rare disorder characterized by severe and protracted diarrhea, weight loss from malabsorption and immune-mediated damage to the intestinal mucosa, generally occurring in infants and young children, only rarely in adult. The salient histopathologic features of AIE are most prominent in the small intestine: villous blunting, crypt hyperplasia, mononuclear cell inflammatory expansion of the lamina propria with intraepithelial lymphocytosis, crypt apoptosis and absence of Paneth cells, goblet cells or both. Esophagus, stomach and colon are frequently also involved. Anti-enterocyte antibodies are identified in the majority of cases, and their presence, even if variable, can help confirming the diagnosis. The purpose of this review is to provide an overview of the latest immunological advances in AIE, as well as to offer a practical approach for histological diagnosis for 'general' pathologist.
Identifiants
pubmed: 34856606
doi: 10.32074/1591-951X-339
pmc: PMC9040544
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
32-39Informations de copyright
Copyright © 2022 Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology.
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