![]() Usually asymptomatic some with recurrent bacterial infections, atopy, and autoimmunity Serum IgA absent or near absent, usually <10 mg/dL normal IgG and IgM levels although IgG2 subclass deficiency may be present impaired specific antibody response in some patients Gene defect unknown defective maturation of B cells into IgA-secreting plasma cells Therefore, it is not surprising that GI disorders are common manifestations, and often the initial presenting symptom, in patients with dysfunction in humoral immunity or cell-mediated immunity ( Table 1). Dysfunction of the regulatory mechanisms maintaining this balance between active immunity and tolerance in the gut may lead to mucosal inflammation and damage and GI diseases. Its response is one of suppression or tolerance, unlike the systemic immune system. The mucosal immune system is uniquely regulated to manage its constant exposure to viruses, parasites, and bacterial antigens, all of which are in close proximity to a large reservoir of lymphocytes, macrophages, and dendritic cells. This is in part because the gut is the largest lymphoid organ in the body, containing the majority of lymphocytes and producing large amounts of immunoglobulin (Ig). Gastrointestinal (GI) disorders present in 5% to 50% of patients with primary immunodeficiencies. The immune defects can affect the humoral (B cell) immune system, such as in Bruton's agammaglobulinemia the cellular (T cell) immune system, such as in DiGeorge syndrome and both T- and B-cell immunity, such as in severe combined immunodeficiency (SCID) 1, 2 and in innate defects. Primary immunodeficiencies are a group of more than 150 disorders, often inherited, that are caused by intrinsic defects in the immune system. This review aims to guide gastroenterologists in the diagnosis and treatment of patients with primary immunodeficiency. Treatment of immunodeficient patients with concomitant gastrointestinal disease can be challenging, and therapy with immunomodulators often is required for severe disease. Therapies for primary immunodeficiency comprise immunoglobulin replacement, antibiotics, and, in severe cases, bone marrow transplantation. Therefore, early recognition of symptoms and referral to an immunologist for a basic immune evaluation is required to select appropriate treatments. However, intestinal biopsy specimens from immunodeficient patients often have distinct histologic features, and these patients often fail to respond to conventional therapies. ![]() Immunodeficient and immunocompetent patients with gastrointestinal diseases present with similar symptoms. Immune-related gastrointestinal diseases can be classified as those that develop primarily via autoimmunity, infection, an inflammatory response, or malignancy. Gastroenterologists therefore must be able to diagnose and treat patients with primary immunodeficiency. The gastrointestinal tract is the largest lymphoid organ in the body, so it is not surprising that intestinal diseases are common among immunodeficient patients. Gastrointestinal disorders such as chronic or acute diarrhea, malabsorption, abdominal pain, and inflammatory bowel diseases can indicate immune deficiency.
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