Gastrointestinal Manifestations and Complications of Primary Immunodeficiency Disorders

Gastrointestinal (GI) involvement can be the presenting disease manifestation in patients with primary immunodeficiency disorders (PIDs). Infections and noninfectious diarrhea are frequent manifestations; however, malignancy and inflammatory and autoimmune-related GI diseases are also described. GI symptoms and disease seen in association with PIDs can mimic other diseases but are often resistant to conventional treatments owing to alternate disease mechanisms. Despite the advances in treatments for these conditions, therapy for immunodeficiency-related GI disease is often empiric.