Surgical Pathology Criteria

IPEX Syndrome (Immune Dysregulation, Polyendocrinopathy, Autoimmune Enteropathy, X-linked)

Differential Diagnosis

 

Tropical Sprue

  • Confined to tropics
  • Responds to anti-microbial therapy

Prolonged Post-enteritis Syndrome

  • History of preceding viral or bacterial gastroenteritis
Celiac Disease Autoimmune Enteropathy
Usually occurs in childhood or older Usually begins under 6 months of age, but can occur in adults
Responds to gluten withdrawal Refractory to gluten withdrawal
Anti-endomysial and - tissue transglutaminase antibodies Anti-enterocyte antibodies
CD4 negative T cells predominate CD4+ T cells predominate
Gamma delta T cell receptors predominate Alpha beta T cell receptors predominate
For pediatric autoimmune enteropathy with associated polyendocrinopathy see IPEX syndrome.
Some adult autoimmune enteropathy cases have been discovered in the workup of patients thought to have celiac disease but who do not respond to removal of gluten from the diet

 

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 Autoimmune Enteropathy
Predominantly eosinophilic infiltrate Predominantly T cell infiltrate
Frequent associated allergy or atopy No association with other allergies or atopy
Resolved by elimination of offending food from diet Not related to diet
For pediatric autoimmune enteropathy with associated polyendocrinopathy see IPEX syndrome.

 

Crohn Disease Autoimmune Enteropathy
Skip lesions predominate Usually diffuse
Usually involves large intestine Principal involvement is duodenum, jejunum and stomach
Transmural inflammation Mucosal inflammation
Granulomas may be seen Granulomas absent
Deep fissures frequent No fissures or deep ulcers
For pediatric autoimmune enteropathy with associated polyendocrinopathy see IPEX syndrome.

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