Surgical Pathology Criteria

Common Variable Immunodeficiency – GI Involvement

Differential Diagnosis

Graft Versus Host Disease

Tropical Sprue

Prolonged Post-enteritis Syndrome

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
The two entities may be histologically indistinguishable.
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

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