Zuzana Havlíčeková, Renata Szépeová, Miloš Jeseňák, Peter Bánovčin
Gastrointestinal tract has primary a barrier function and it is the largest lymphoid organ in the body. So, it is not surprising that gastrointestinal and hepatobiliary manifestations are common and often the initial presenting problem in the patients with underlying primary immunodeficiency disorders. They may even precede the primary immunodeficiency diagnosis in some cases. The risk and severity of gastrointestinal complications depend on the type of immunodeficiency. Patients with humoral and combined immunodeficiency are at higher risk to develop gastrointestinal manifestations. Gastrointestinal complications result from five different factors: infection, autoimmunity, unregulated inflammation, malignancy and complications of therapeutic interventions. Many of disorders mimic classic forms of the disease (in absence of immunodeficiency), such as celiac sprue or inflammatory bowel diseases. Patients presenting with atypical gastrointestinal symptoms or common gastrointestinal symptoms and failure to respond to conventional therapy should be evaluated for an underlying primary immune disorder. Early diagnosis, adequate treatment and nutritional support improve quality of life and prevent long-term complications.