A Case of Celiac Crisis Averted

Abstract

Purpose: A 56-year-old male smoker with hypertension and hyperlipidemia was seen in the GI clinic (July 2010) for 6 weeks of new onset crampy, watery diarrhea without blood or mucus and regardless of food. His diarrhea occasionally awakens him from sleep; he has lost 20 lbs and is fatigued. The differential included infectious, inflammatory, autoimmune diarrhea and celiac disease. He was repeatedly hospitalized (at one point over 60 days continuously) September 2010-January 2011 following diverticulitis requiring colostomy. He was empirically placed on a gluten-free diet with no improvement. His post-colostomy output was up to 30 L/day lasting for weeks. The cause was elusive. During this time, he required TPN, aggressive resuscitation, electrolyte replacement, and meanwhile, an extensive work-up yielded normal results with no evidence of infection, autoimmunity, or hormonal abnormalities (TTG, anti-EMA, metabolite derangement, hormone/enzyme abnormality, or dietary deficiency/excess). ESR, CRP, and pancreatic polypeptide were mildly elevated; DQ2 was positive and DQ8 was negative. In addition, he had multiple endoscopies with small and large bowel biopsies that ultimately led to the diagnosis of celiac disease with crisis. With the diagnosis of celiac crisis finally made, he was aggressively treated with high dose IV steroids and he dramatically improved. He thus continued on gluten-free diet and steroids. Discussion: Celiac Crisis (which can include severe diarrhea, dehydration, metabolic disturbances, neurologic and renal dysfunction, hypoproteinemia, and weight loss) is the fulminant form of celiac disease. It is rare and difficult to diagnose, but should be considered in cases of significant diarrhea of uncertain cause. The etiology is unclear; however, it is likely a combination of mucosal inflammation, immune mediation, and disruption of the normal patterns of motility. Crisis is often precipitated by infection, surgery, pregnancy, or other immune mediating event (surgery in this case). Our patient responded well; he is an outpatient on a slow taper of prednisone, complying with a gluten-free diet, and has normal colostomy output and electrolytes - Crisis Averted.