S2030 Circulating Hematopoietic Stem Cells and Dynamics of Intestinal Regeneration Following Gluten-Free Diet in Celiac Patients

Abstract

Purpose: Celiac crisis is a life-threatening syndrome where celiac disease presents with profuse diarrhea and severe metabolic disturbances. Celiac crisis in adults is believed to be uncommon and is not well documented. However, it is likely that many patients with celiac crisis are not definitively diagnosed. In order to improve awareness and to facilitate diagnosis, we reviewed cases of celiac crisis seen at Beth Israel Deaconess Medical Center in Boston, MA and Mayo Clinic in Rochester, MN. These data were used to identify presenting features, formulate diagnostic criteria for celiac crisis in adults and develop treatment strategies. Methods: We reviewed cases of biopsy proven celiac disease in the last 5 years. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms attributable to celiac disease requiring hospitalization and/or parenteral nutrition along with at least 2 of the 7 factors listed below: 1.Signs of severe dehydration including hemodynamic instability or orthostasis 2.Neurologic dysfunction 3.Renal dysfunction: creatinine >2.0 4.Metabolic acidosis: pH 10 lbs Results: Twelve patients met the above criteria. Of these, 8 were women and 4 were men. 11 developed celiac crisis prior to diagnosis of celiac disease. IgA tissue transglutaminase titer was available for 11 patients. Of these, one had IgA deficiency, and all of the others had elevated tTG, eight with levels greater than 4 times the normal limits. Biopsies of the duodenum in all patients were consistent with a Marsh 3 score. HLA type was available for 10, of which 9 were positive for DQ2 and 1 for DQ8. Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances, of which hypocalemia was most common. All patients required hospitalization and intravenous fluids and five required parenteral nutrition. Six patients required corticosteroids. All patients had a rapid clinical response to gluten free diet. Conclusion: Celiac crisis is associated with high morbidity and although rarely described, does occur in adults, often without a clear precipitating factor. Patients presentingwith severe unexplained diarrhea and malabsorption should be tested for celiac disease and treatment with systemic steroids or oral budesonide considered. Nutritional support is often required in the short term but most patients ultimately respond to gluten avoidance. We are hopeful that our delineation of formal diagnostic criteria for celiac crisis will aid in identification and management of these patients.