Life-threatening onset of coeliac disease: a case report and literature review

Abstract

BackgroundCoeliac 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.MethodsThis 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.ResultsIn 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.ConclusionsThe 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.

Effect of Gluten-Free Diet on Gut Microbiota Composition in Patients with Celiac Disease and Non-Celiac Gluten/Wheat Sensitivity

“…Since the enterocyte loss is not sufficiently compensated by a rapid stem cell-derived epithelial replacement, crypt hyperplasia and villous atrophy occur. Moreover, when gliadin peptides get across the altered epithelial lining and reach the bloodstream, they enhance inflammation, thus spreading the immune response and causing extraintestinal manifestations [ 26 , 27 ]. The ubiquitous distribution of TG2, recognized as the main CD autoantigen, along with the increased intestinal permeability (i.e., leaky gut) makes CD a unique immunological systemic disorder favoring the onset of an autoimmune response also in other tissues and organs [ 6 , 28 , 29 , 30 , 31 , 32 ].…”

Small and Large Intestine (I): Malabsorption of Nutrients

“…This would imply that even a typically chronic disorder, such as CeD, may have acute onset in a small proportion of patients, which emergency physicians should be aware of. Although rarely encountered in clinical practice, this acute onset of CeD requires hospitalization and immediate treatment (i.e., electrolyte replacement and protein correction) to avoid life-threatening complications [ 179 ].…”

Probiotics, Prebiotics and Other Dietary Supplements for Gut Microbiota Modulation in Celiac Disease Patients

“…To date, the unique treatment that has proven to be effective in CD patients is strictly life-long adherence to the gluten-free diet (GFD), i.e., a diet containing less than 10 mg per day of gluten [1,2]. Lack of adherence to the GFD leads to a significant risk of adverse health consequences [5] and increases mortality from malignancies (e.g., small bowel adenocarcinoma, cancer of the esophagus, B-cell and T-cell non-Hodgkin lymphomas), and in particular intestinal T-cell lymphomas; the risk reduces with strict adherence to the diet [6,7]. Despite rigid compliance to GFD, several studies showed that patients struggle in achieving a full restoring of the gut microbiota; the cause of this phenomenon could be inferred from a persistent genetic influence than to the lack of prebiotics usually ingested with gluten assumption [8].…”