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Life-threatening onset of coeliac disease: a case report and literature review
  1. Matteo Guarino1,
  2. Edoardo Gambuti1,
  3. Franco Alfano1,
  4. Andrea Strada2,
  5. Rachele Ciccocioppo3,
  6. Lisa Lungaro1,
  7. Giorgio Zoli1,
  8. Umberto Volta4,
  9. Roberto De Giorgio1,
  10. Giacomo Caio1,5
  1. 1Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara, Italy
  2. 2Department of Emergency Medicine, St. Anna University Hospital, Ferrara, Italy
  3. 3Department of Medicine, A.O.U.I. Policlinico G.B. Rossi and University of Verona, Verona, Italy
  4. 4Department of Medical and Surgical Sciences, University of Bologna, Bologna, italy
  5. 5Celiac Center and Mucosal Immunology and Biology Reaserch Center, Massachusetts General Hospital - Harvard Medical School, Boston, MA, United States
  1. Correspondence to Dr Giacomo Caio; caigmp{at}


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.

  • diarrhoea
  • gluten-free diet
  • malabsorption
  • intestinal failure
  • coeliac disease

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  • Contributors MG, RDG and GC: design and conceptualisation. MG, EG and FA reviewed the literature and wrote the first draft of the manuscript. AS, RC, LL, GZ, UV, RDG and GC participated in the clinical assessment of the patient and critically reviewed the paper.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent for publication Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data availability statement Data sharing not applicable as no data sets generated and/or analysed for this study.

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