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COMMON VARIABLE IMMUNODEFICIENCY-ASSOCIATED DIARRHEA AS MIMICKER TO GLUTEN-SENSITIVE ENTEROPATHY
E Ching, A MacPherson, R McLoughlinMcMaster University, Hamilton, Ontario
Aims: Common variable immunodeficiency (CVID) is a primary antibody deficiency characterized by hypogammaglobulinemia with impaired antibody response leading to recurrent infections with respiratory and GI tracts being most commonly involved, and a greater incidence of autoimmune and neoplastic diseases. Up to 60% of untreated CVID patients develop diarrhea and 10% develop idiopathic malabsorption-associated weight loss. We report on a patient with CVID and chronic diarrhea with biopsy suggestive of gluten sensitive enteropathy (celiac sprue) who significantly improved with budesonide.
Methods: A 50 yr-old woman with history of long standing type 1 diabetes and CVID receiving intravenous immunoglobulin (IVIG) presents with progressive diarrhea with significant weight loss.
Results: Her gastric biopsy showed chronic active gastritis and duodenal biopsies showed villous atrophy and intraepithelial lymphocytes consistent with celiac disease. Her celiac serology was negative but on a background of low immunoglobulin levels. Her small bowel meal showed diffuse thickening of small bowel folds suggestive of enteropathy. She failed to respond to a gluten free diet. She had moderate improvement with a trial of high dose prednisone but relapsed as the doses were tapered down. She then developed intolerance to high dose prednisone. Her stool cultures, oval and parasites, and virology were negative. Her CT abdomen/pelvic did not show any neoplasm. Her colonic biopsies showed mild crypt distortion and mild diffuse intra-epithelial lymphocytes suggestive of lymphocytic colitis. Her lactose breath test was positive but she failed to respond to cyclical antibiotics for bacterial overgrowth, and she developed hypokalemia and hypoalbuminemia with significant ascites and edema. Her biopsies were reviewed again, while they suggested celiac disease and lymphocytic colitis, they were also consistent with CVID-associated chronic GI inflammation. She responded well to a trial of budesonide with gradual weight gain and resolution of her diarrhea, ascites and edema.
Conclusions: Bacterial overgrowth is a well recognized problem in CVID, but our patient failed to respond to cyclical antibiotics. Literature has shown that up to 20% of CVID patients have chronic active gastritis and up to 25% of them have sprue-like disease, and they are more often associated with symptomatic patients. IVIG has been the mainstay treatment for CVID to restore immunoglobulin levels, but it has minimal effect on CVID-associated chronic diarrhea. In fact, studies have shown that CVID patients with GI manifestations have abnormal interleukins and cytokines response suggestive more defective T-cell function than antibody deficiency. Therefore, locally acting corticosteroid such as budesonide may be clinically effective in treating these patients.