TY - JOUR T1 - Life-threatening onset of coeliac disease: a case report and literature review JF - BMJ Open Gastroenterology JO - BMJ Open Gastro DO - 10.1136/bmjgast-2020-000406 VL - 7 IS - 1 SP - e000406 AU - Matteo Guarino AU - Edoardo Gambuti AU - Franco Alfano AU - Andrea Strada AU - Rachele Ciccocioppo AU - Lisa Lungaro AU - Giorgio Zoli AU - Umberto Volta AU - Roberto De Giorgio AU - Giacomo Caio Y1 - 2020/05/01 UR - http://bmjopengastro.bmj.com//content/7/1/e000406.abstract N2 - 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. ER -