Celiac Crisis: A Rare Presentation of Celiac Disease in Adults

Abstract

Background: Celiac Disease is an immune-mediated enteropathy resulting from an inappropriate and exaggerated response to dietary gluten [1, 2]. Celiac Crisis is an un-common, life-threatening condition resulting from acute severe diarrheal illness, severe metabolic disturbances, and malabsorption that is usually found in children, with only 22 cases reported in adults [3]. Here we report a case of celiac crisis in a middle aged male with characteristic endoscopic and pathological findings. Case Report: A 58-year-old male with previous history of left facial crush injury necessitating several reconstructive surgeries in 2012 presented with profuse, watery, foul-smelling diarrhea of 2 months duration with episodic fatigue, melena, and 20 kg weight loss. Since his accident in 2012 he had intermittent episodes of loose stools that got significantly worse within 2 months. He had also been diagnosed with Clotridium difficle colitis and received 14 days of oral antibiotics one month prior to his presentation. Physical exam revealed pallor and cachexia. Laboratory findings showed hemoglobin of 4.7 g/dL, INR greater than 10 (without anticoagulation), albumin 1.7 g/dL, potassium 2.9 mmol/L, undetectable levels of fat soluble vitamins, and negative stool studies. Esophagogastroduodenoscopy revealed diffuse granular mucosa in the duodenum with fissuring and scalloping appearance [Fig 1]. Pathologic examination showed severe villous atrophy and hypoplasia (March IV) with increased intra-epithelial lymphocytes [Fig 2]. IgA anti-tissue transglutaminase antibodies titers were >200 U/mL (normal, 0-19 U/mL). He was diagnosed with celiac disease presenting with celiac crisis and was started on a gluten-free diet with vitamin supplementations, following which he made a full recovery.Figure 1Figure 2Discussion: Celiac crisis has been described in association with or following surgery, pregnancy, immunosuppressive therapy, and infection. The probable triggering factor in our patient was clostridium difficle infection. However, another association can also be made between the patient's head trauma and triggering of celiac disease. This link was investigated by a Swedish study, which found a very small excess risk for future celiac disease in individuals with an earlier head trauma [4].Figure 3