Table 1 Primary immunodeficiency disorders with gastrointestinal manifestations
From: Inflammatory and infectious manifestations of immunodeficiency in the gastrointestinal tract
Feature | Common variable immunodeficiency | Selective IgA deficiency | Chronic granulomatous disease |
|---|---|---|---|
Immunologic defect | Hypogammaglobulinemia | Decreased mucosal immunity | Impaired destruction of phagocytosed microorganisms |
Key histologic features | Chronic active colitis with crypt architectural distortion. Variably severe villous defect. Absent or decreased plasma cells. More pronounced intraepithelial inflammation in deep mucosa compared with surface. Lymphoid hyperplasia. Increased apoptosis in glands and crypts. Lymphocytic or collagenous colitis pattern of inflammation.Granulomata. | May appear normal. Shortened small intestinal villi with crypt hyperplasia. Intraepithelial lymphocytosis. Lymphoid hyperplasia. | Shortened small intestinal villi. Chronic active colitis with crypt architectural distortion. Granulomata. Pigmented lamina propria macrophages. |
Associated gastrointestinal disorders | Cytomegalovirus, Cryptosporidium. Salmonella spp., Giardia lamblia infections. Esophageal candidiasis. Extranodal B-cell lymphomas. | Giardia lamblia and Strongyloides infections. Celiac disease. Inflammatory bowel disease. Gastric adenocarcinoma. | Invasive fungal infections, particularly Aspergillus spp. Bacterial infections, particularly Salmonella spp. |
