Celiac Crisis: A Rare Manifestation of a Common Disease

Abstract

Introduction: Celiac crisis is a rare and life threatening manifestation of celiac disease. Case presentation: We present a 46 year old male with a history of DM1 complicated by ESRD on HD and a recent diagnosis of Bell's Palsy who was admitted from a dialysis center with hypotension and voluminous diarrhea. Reported up to 20 watery, nonbloody bowel movements per day x 7 days. The diarrhea was associated with LLQ crampy pain. Denied fevers. +Nocturnal diarrhea. The diarrhea did not improve with fasting. Denied recent weight loss.Figure 1Figure 2Figure 3Vital Signs and PE: Afebrile BP 55/42 Pulse 102. Dry mucous membranes. Mild LLQ tenderness with hyperactive bowel sounds. A rectal tube was in place with voluminous light brown watery stool identified. Inital laboratory results and imaging. 6.8/11\173. 130 | 98 | 98. 4.6 | 19 | 13.4. pH 7.27 Alb 2.9 Ca 7.2 C. Diff PCR negative CT abdomen and pelvis with IV contrast revealed mild thickening of proximal small bowel loops, suspicious for enteritis. Further pertinent labs: Stool 0 + P and culture negative. Lactoferrin: positive. ESR and CRP elevated. TTG IgA 80 (0-19). Gliadin IgA 39 (0-19). Stool Osmolar gap 36 Gastrin and VIP normal 5HIAA excretion normal. Over a 24 hour period, the patient required 13 L's of IVF's to keep up with GI losses and maintain blood pressure. An esophagogastroduodenoscopy revealed scalloping in the duodenal bulb and 2nd part of the duodenum. Biopsies were obtained and revealed villous atrophy, increased intraepithelial lymphocytes, and crypt hyperplasia (Marsh 3C). The diagnosis of celiac crisis was made based on the endoscopic and serologic results in conjuction with the dramatic presentation. After 3 days of gluten withdrawal our patients bowel movement frequency and caliber improved dramatically. Discussion: In this rare manifestation of celiac disease marked metabolic derangements are encountered. It is unclear what triggers this more aggressive disease course, however it has been suggested to be a combination of severe mucosal inflammation and immune activation.1 In our case, we propose that his recent diagnosis of Bell's Palsy precipitated an immune activation leading to the crisis. Celiac disease should be considered in adults presenting with acute voluminous diarrhea and profound electrolyte abnormalities.