CLINICAL AND HISTOLOGIC IMPROVEMENT OF ENTEROPATHY ASSOCIATED WITH COMMON VARIABLE IMMUNODEFICIENCY WITH BUDESONIDE THERAPY

Abstract

Common variable immunodeficiency (CVID) is an immunologic disorder characterized by defective antibody production. CVID has been associated with an enteropathy that has similarities to gluten-sensitive enteropathy with villous atrophy in the small bowel resulting in malabsorption. It does not respond to gluten avoidance and there are no proven therapies, though use of oral prednisone has been described. However, long-term immunosuppressive therapy in CVID might increase a patient's risk of infectious and systemic complications. This report describes an alternative approach to improve symptoms and histology in CVID enteropathy. Case: A 41 year-old male with CVID presented with progressive diarrhea and weight loss, describing up to 20 steatorrheic bowel movements a day over the past year. He denied fevers, chills, hematochezia, or other systemic complaints, excepting a 30 pound weight loss. His only significant medical problem was CVID, treated with regular IV immunoglobulin (IVIG) infusions for the previous three years. The patient took no medications and denied recent travel or sick contacts. Examination showed wasting, but was otherwise normal. Labs showed evidence of malabsorption and malnutrition. All stool studies were negative. Transglutaminase Ab. was negative, but small bowel biopsy revealed villous blunting. Colonoscopy and abdominal CT scan were normal. Though empirically started on a gluten-free diet, symptoms and villous blunting persisted. The patient was diagnosed with CVID enteropathy while on IVIG, so prednisone was added to treat the enteropathy-improving both his diarrhea and villous blunting. When the prednisone was tapered below 10 mg per day, the diarrhea and villous blunting recurred. So, prednisone with oral budesonide was started. The patient was weaned off prednisone over 3 months, remaining solely on oral budesonide for the past year without recurrence of diarrhea. Prealbumin and body weight have normalized. Villous blunting has improved. Discussion: There are limited therapeutic options for CVID enteropathy. Given the risks associated with long-term systemic steroids, this case report defines a viable Treatment option. Treatment of CVID enteropathy with oral budesonide has not been previously published.