2640 Intractable Celiac Disease in IgA Deficiency

Abstract

INTRODUCTION: Celiac disease is an immune-mediated disorder triggered by a reaction to gluten with elevated levels of IgA antibodies. Symptoms typically resolve with a gluten free diet. However, refractory cases have been reported despite dietary adherence. Our discussion involves an elderly woman with severe, refractory celiac disease with concomitant IgA deficiency. CASE DESCRIPTION/METHODS: An 80-year-old woman presented our clinic with exacerbation of chronic, non-bloody diarrhea. She has a history of celiac disease diagnosed 20 years ago after an episode of acute pancreatitis. She also has IgA deficiency and her total IgA level remains low at 37, despite infusions 3 times per week. She is strictly adherent to a gluten free diet, but continues to have multiple episodes of diarrhea and has lost over 100 pounds since her diagnosis. Patient had bidirectional endoscopy notable for antral gastropathy as well as fissures and flattening of the duodenal mucosa. Biopsies were consistent with celiac sprue. Capsule endoscopy showed diffuse flattening of the small bowel villi with erythema and cracked, scalloped mucosa as well as multiple, small, aphthous ulcers throughout the bowel (Figures 1, 2, and 3). Patient has had an extensive workup, but everything else have been unrevealing. Steroids have been initiated as a last-ditch effort, but she continues to have “more bad days than good days” despite being on a high dose prednisone. DISCUSSION: The diagnosis and treatment of celiac disease can be complicated by coexisting immunodeficiencies. Usually, titers of tissue transglutaminase IgA will be elevated and are involved in the immune reaction that causes damage to the lining of the small bowel with characteristic flattening of the villi as well as chronic diarrhea and malabsorption. A connection between IgA deficiency and celiac disease has been well established and likely contributes to the refractory nature of our patient’s disease. In patients with IgA deficiency it is important to utilize other forms of serologic testing, typically with IgG tissue transglutaminase, in addition to duodenal biopsies. Symptoms typically resolve with a gluten free diet. However, refractory cases have been reported with persistent diarrhea and villous atrophy for over 1–2 years. Symptom management can be difficult for these patients and may require the use of steroids as the last resource.