Gastrointestinal Manifestations and Complications of Primary Immunodeficiency Disorders

Gastrointestinal (GI) involvement can be the presenting disease manifestation in patients with primary immunodeficiency. Diarrhea both infections and non-infectious is the most frequent manifestation, but malignancy, inflammatory and autoimmune related GI diseases are also described. GI symptoms and pathology seen in association with PID can mimic other disease, such as celiac sprue and IBD but, are often resistant to conventional treatments owing to alternate disease mechanisms. Despite the advances in treatments for these inflammatory conditions, therapy for immunodeficiency related GI disease is often empiric.