Life-threatening onset of coeliac disease: a case report and literature review.
Adult
Aged
Atrophy
/ diagnosis
Biopsy
Celiac Disease
/ complications
Diet, Gluten-Free
/ methods
Duodenum
/ pathology
Female
GTP-Binding Proteins
/ immunology
HLA-DQ Antigens
/ metabolism
Haplotypes
Humans
Hypoproteinemia
/ etiology
Immunoglobulin A
/ immunology
Malabsorption Syndromes
/ etiology
Male
Middle Aged
Mucous Membrane
/ pathology
Protein Glutamine gamma Glutamyltransferase 2
Severity of Illness Index
Transglutaminases
/ immunology
Treatment Outcome
coeliac disease
diarrhoea
gluten-free diet
intestinal failure
malabsorption
Journal
BMJ open gastroenterology
ISSN: 2054-4774
Titre abrégé: BMJ Open Gastroenterol
Pays: England
ID NLM: 101660690
Informations de publication
Date de publication:
05 2020
05 2020
Historique:
received:
19
03
2020
revised:
07
04
2020
accepted:
08
04
2020
entrez:
9
5
2020
pubmed:
10
5
2020
medline:
20
7
2021
Statut:
ppublish
Résumé
Coeliac disease (CD) results from an immune-mediated reaction to gluten in genetically predisposed individuals. In rare cases CD may occur with acute features deferring the diagnosis and exposing these patients to possible life-threatening complications. Herein we present the case of a young woman with a coeliac crisis, that is, a sudden clinical onset characterised by severe electrolyte imbalance due to an unknown (previously unrecognised) CD. This is a case report and literature review revealing that coeliac crisis is under-reported, with a total of 48 adult cases so far published. The diagnosis in our case was established by histopathological analysis of multiple duodenal biopsies. The patient's serum was tested by enzyme-linked immunoassay to detect antitransglutaminase IgA antibodies. In contrast to cases reported in the literature, with male gender predominance and a mean age of 50±17 years, our patient was a young female case of coeliac crisis. However, like in our patient, a higher incidence of coeliac crisis was associated with the human leucocyte antigen (HLA)-DQ2 haplotype, versus HLA-DQ8, and a severe (Marsh-Oberhüber 3c) duodenal mucosa atrophy. Notably, there is no clear correlation between the antitissue transglutaminase 2 IgA antibody titre and coeliac crisis onset/severity, as confirmed by our case report. The present case highlights that CD may manifest quite abruptly with a severe malabsorption syndrome, that is, electrolyte abnormalities and hypoproteinaemia. Our case should alert physicians, in particular those in the emergency setting, that even a typically chronic disorder, such as CD, may show life-threatening complications requiring urgent management.
Sections du résumé
BACKGROUND
Coeliac disease (CD) results from an immune-mediated reaction to gluten in genetically predisposed individuals. In rare cases CD may occur with acute features deferring the diagnosis and exposing these patients to possible life-threatening complications. Herein we present the case of a young woman with a coeliac crisis, that is, a sudden clinical onset characterised by severe electrolyte imbalance due to an unknown (previously unrecognised) CD.
METHODS
This is a case report and literature review revealing that coeliac crisis is under-reported, with a total of 48 adult cases so far published. The diagnosis in our case was established by histopathological analysis of multiple duodenal biopsies. The patient's serum was tested by enzyme-linked immunoassay to detect antitransglutaminase IgA antibodies.
RESULTS
In contrast to cases reported in the literature, with male gender predominance and a mean age of 50±17 years, our patient was a young female case of coeliac crisis. However, like in our patient, a higher incidence of coeliac crisis was associated with the human leucocyte antigen (HLA)-DQ2 haplotype, versus HLA-DQ8, and a severe (Marsh-Oberhüber 3c) duodenal mucosa atrophy. Notably, there is no clear correlation between the antitissue transglutaminase 2 IgA antibody titre and coeliac crisis onset/severity, as confirmed by our case report.
CONCLUSIONS
The present case highlights that CD may manifest quite abruptly with a severe malabsorption syndrome, that is, electrolyte abnormalities and hypoproteinaemia. Our case should alert physicians, in particular those in the emergency setting, that even a typically chronic disorder, such as CD, may show life-threatening complications requiring urgent management.
Identifiants
pubmed: 32381744
pii: bmjgast-2020-000406
doi: 10.1136/bmjgast-2020-000406
pmc: PMC7223027
pii:
doi:
Substances chimiques
HLA-DQ Antigens
0
HLA-DQ2 antigen
0
Immunoglobulin A
0
Protein Glutamine gamma Glutamyltransferase 2
EC 2.3.2.13
Transglutaminases
EC 2.3.2.13
GTP-Binding Proteins
EC 3.6.1.-
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.
Déclaration de conflit d'intérêts
Competing interests: None declared.
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