BACKGROUND: Celiac crisis is an uncommon but critical complication of celiac disease (CD) manifesting with copious diarrhea, dehydration, and severe metabolic imbalances. Celiac crisis occurring in individuals who have been formerly diagnosed with CD and displaying severe coagulopathy is tremendously rare.

CASE REPORT: We report a case of a 76-year-old male, previously diagnosed with CD and non-compliant with gluten free diet, who presented with severe coagulopathy manifesting as gastrointestinal bleeding in addition to other features of celiac crisis, including severe diarrhea, dehydration, metabolic acidosis, electrolyte disturbances, and renal dysfunction. Esophagogastroduodenoscopy revealed flattened mucosa and mucosal nodularity in the duodenum. Duodenal biopsies exhibited active chronic inflammation with intraepithelial lymphocytosis and subtotal villous blunting. The patient was diagnosed with celiac crisis and treatment with vitamin K, parenteral nutrition, and steroids was commenced. After initial clinical improvement, a gluten-free diet was implemented with complete resolution of symptoms.

CONCLUSIONS: Though celiac crisis typically presents in patients with undiagnosed CD, it should be considered in patients who have been previously diagnosed CD but who are non-compliant with gluten free diet. Severe coagulopathy, though extremely rare, can be a feature of celiac crisis and should be consider when encountered in a patient with history of steatorrhea and gastrointestinal bleeding.

Keywords: celiac disease, Dehydration, malnutrition, Vitamin K Deficiency Bleeding, Weight Loss, Blood Coagulation Disorders, Diet, Gluten-Free, Patient Compliance