Common Variable Immunodeficiency – GI Involvement
Differential Diagnosis
- Graft versus host disease
- Tropical sprue
- Celiac disease
- Autoimmune enteropathy
- Food protein enteropathy
- Crohn disease
Graft Versus Host Disease
- The gastrointestinal findings in common variable immunodeficiency may be histologically indistinguishable from graft versus host disease
- They are easily distinguished by a history of transplantation in the latter
Tropical Sprue
- Confined to tropics
- Responds to anti-microbial therapy
- Plasma cells are present
Prolonged Post-enteritis Syndrome
- Preceding viral or bacterial gastroenteritis
Autoimmune Enteropathy | Common Variable Immunodeficiency – GI Involvement |
Numerous plasma cells | Plasma cells frequently markedly decreased |
Diffuse infiltrate primarily composed of T lymphocytes | Prominent nodular lymphoid hyperplasia |
Prominent villous atrophy | Villous atrophy may be mild to severe |
Prominent crypt destruction | Crypt destruction not prominent |
Anti-enterocyte antibody | All antibodies generally depressed; humoral testing is not reliable in CVID |
For pediatric autoimmune enteropathy with associated polyendocrinopathy see IPEX syndrome.
Food Protein Enteropathy | Common Variable Immunodeficiency – GI Involvement |
Numerous plasma cells | Plasma cells frequently markedly decreased |
Predominantly eosinophilic infiltrate | Nodular lymphoid hyperplasia |
Frequent associated allergy or atopy | No association with other allergies or atopy |
Resolved by elimination of offending food from diet | Not related to diet |
Crohn Disease | Common Variable Immunodeficiency – GI Involvement |
Skip lesions predominate | Usually diffuse |
Transmural inflammation | Mild lamina propria infiltrate with nodular lymphoid hyperplasia |
Numerous plasma cells | Plasma cells frequently markedly decreased |
Deep fissures frequent | No fissures or deep ulcers |