TY - JOUR
T1 - Celiac Crisis Is a Rare but Serious Complication of Celiac Disease in Adults
AU - Jamma, Shailaja
AU - Rubio-Tapia, Alberto
AU - Kelly, Ciaran P.
AU - Murray, Joseph
AU - Najarian, Robert
AU - Sheth, Sunil
AU - Schuppan, Detlef
AU - Dennis, Melinda
AU - Leffler, Daniel A.
N1 - Funding Information:
Funding This work was supported in part by the National Institutes of Health under the Ruth L. Kirschstein National Research Service Award/Training grant in Gastrointestinal Allergy and Immunology Research ( T32 AI07047 to A.R.-T.) and National Institutes of Health grant ( DK57892 to J.A.M.) as well as by internal funding from the Celiac Center at Beth Israel Deaconess Medical Center .
PY - 2010/7
Y1 - 2010/7
N2 - Background & Aims: Celiac crisis is a life-threatening syndrome in which patients with celiac disease have profuse diarrhea and severe metabolic disturbances. Celiac crisis is rare among adults and not well documented. To improve awareness of this condition and to facilitate diagnosis, we reviewed cases of celiac crisis to identify presenting features, formulate diagnostic criteria, and develop treatment strategies. Methods: Cases of biopsy-proven celiac disease were reviewed. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms that could be attributed to celiac disease and required hospitalization and/or parenteral nutrition, along with signs or symptoms of dehydration or malnutrition. Results: Twelve patients met preset criteria for celiac crisis; 11 developed celiac crisis before they were diagnosed with celiac disease. Eleven patients had increased titres of transglutaminase antibodies and 1 had immunoglobulin A deficiency. Results of biopsy analyses of duodenum samples from all patients were consistent with a Marsh 3 score (33% with total villous atrophy). Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances. All patients required hospitalization and intravenous fluids, 6 required corticosteroids, and 5 required parenteral nutrition. All patients eventually had a full response to a gluten-free diet. Conclusions: Celiac crisis has a high morbidity and, although rarely described, occurs in adults and often has a clear precipitating factor. Patients who present with severe unexplained diarrhea and malabsorption should be tested for celiac disease; treatment with systemic steroids or oral budesonide should be considered. Nutritional support often is required in the short term but most patients ultimately respond to gluten avoidance.
AB - Background & Aims: Celiac crisis is a life-threatening syndrome in which patients with celiac disease have profuse diarrhea and severe metabolic disturbances. Celiac crisis is rare among adults and not well documented. To improve awareness of this condition and to facilitate diagnosis, we reviewed cases of celiac crisis to identify presenting features, formulate diagnostic criteria, and develop treatment strategies. Methods: Cases of biopsy-proven celiac disease were reviewed. Celiac crisis was defined as acute onset or rapid progression of gastrointestinal symptoms that could be attributed to celiac disease and required hospitalization and/or parenteral nutrition, along with signs or symptoms of dehydration or malnutrition. Results: Twelve patients met preset criteria for celiac crisis; 11 developed celiac crisis before they were diagnosed with celiac disease. Eleven patients had increased titres of transglutaminase antibodies and 1 had immunoglobulin A deficiency. Results of biopsy analyses of duodenum samples from all patients were consistent with a Marsh 3 score (33% with total villous atrophy). Patients presented with severe dehydration, renal dysfunction, and electrolyte disturbances. All patients required hospitalization and intravenous fluids, 6 required corticosteroids, and 5 required parenteral nutrition. All patients eventually had a full response to a gluten-free diet. Conclusions: Celiac crisis has a high morbidity and, although rarely described, occurs in adults and often has a clear precipitating factor. Patients who present with severe unexplained diarrhea and malabsorption should be tested for celiac disease; treatment with systemic steroids or oral budesonide should be considered. Nutritional support often is required in the short term but most patients ultimately respond to gluten avoidance.
KW - Enteropathy
KW - Steroids
KW - Tissue Transglutaminase
KW - Treatment
UR - http://www.scopus.com/inward/record.url?scp=77953683769&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77953683769&partnerID=8YFLogxK
U2 - 10.1016/j.cgh.2010.04.009
DO - 10.1016/j.cgh.2010.04.009
M3 - Article
C2 - 20417725
AN - SCOPUS:77953683769
SN - 1542-3565
VL - 8
SP - 587
EP - 590
JO - Clinical Gastroenterology and Hepatology
JF - Clinical Gastroenterology and Hepatology
IS - 7
ER -