Introduction: Celiac crisis is an uncommon presentation in adults. While serology is sensitive and specific, it can be negative in some patients, which make intestinal biopsy indispensable in diagnosis. An 80-year-old woman presented to the hospital with loose stools; 15 episodes /day for 1 week, and increasing weakness. She had 10-year history of 3-4 loose stools/day. She has no significant past medical or family history, nor recent travel history. Labs showed low sodium, potassium, chloride, metabolic acidosis, and acute renal failure. She was initially treated with intravenous normal saline and bicarbonate; however, diarrhea continued. Investigations showed elevated stool osmotic gap and negative stool cultures for bacteria, ova, and parasites. Anti-endomysial (EMA) and anti-tissue transglutaminase (TTG) antibodies and 5HIAA were normal. Colonoscopy demonstrated normal colonic mucosa; however, random biopsies demonstrated lymphocytic colitis and increased intraepithelial lymphocytes. Small bowel biopsy showed partial villous atrophy, crypt hyperplasia, and increased inflammatory infiltrate consistent with celiac disease. She was started on a gluten-free diet, and over the subsequent weeks, she had resolution of symptoms. Two interesting aspects of this case are that she presented with celiac crisis, which is rare in adults, and diagnosis depended solely on biopsy because she did not have antibodies to EMA and TTG. Celiac crisis is a life-threatening syndrome of unclear etiology. Manifestations include diarrhea and electrolyte disturbances. It was thought to occur exclusively in patients younger than 2 years of age. The association between microscopic colitis and celiac disease may be 1 factor that contributes to the development celiac crisis due to defective active and passive absorption of sodium and chloride, and reduced chloride-bicarbonate exchange. Diagnosis of celiac disease is based on serologic tests of specific antibodies, including EMA and TTG antibodies, which are highly specific and sensitive (95% and 96%). It was found that 7% of patients were negative for both auto-antibodies despite having normal IgA levels. Intestinal biopsy is the gold standard for diagnosis because EMA and TTG antibodies decrease in patients with partial villous atrophy. Histological criteria are increased intraepithelial lymphocyte infiltrate, crypt hyperplasia, and villous atrophy. Gluten-free diet is the mainstay of treatment with dramatic response and improvement in symptoms. Although celiac crisis is an uncommon presentation in adults, it should be considered in patients presenting with unexplained severe diarrhea and metabolic acidosis, even if EMA and TTG antibodies are negative.
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