CVID - Patients are at risk for developing a number of GI tract pathologies including lymphoid nodular hyperplasia and inflammatory bowel disease. An enteropathy with villous blunting (resembling celiac disease) may be present but does not respond to dietary gluten removal. The risk of developing GI tract malignancies (lymphoma, adenocarcinoma) is increased 50 fold.y.
IPEX syndrome - Patients develop intractable chronic diarrhea secondary to autoimmune small bowel inflammation (the colon can also be affected). The diarrhea may be watery, bloody or mucoid. Biopsy of the bowel reveals villous atrophy and lymphocytic infiltrates in the mucosa. Additional features of this disease include atopic dermatitis, endocrinopathies (Type I diabetes and thyroid disease), and autoimmune cytopenias.