Primary immunodeficiencies represent a group of inherited disorders of immunity system.The main phenotypic features account for infections, malignity, allergy, autoimmunity and autoinflammatory complications. Treatment and prognosis depend on the time of diagnosis, type of genetic defect and severity of a clinical manifestation.
The article describes gastrointestinal complications in patients suffering from humoral immunodeficiencies (selective IgA deficiency, common variable immunodeficiency) and some recent clinical entities such as activated phosphoinositide 3-kinase delta syndrome, CTLA-4 deficiency and LRBA deficiency.