Diagnosis and Treatment of Gastrointestinal Disorders in Patients With Primary Immunodeficiency

Abstract

Gastrointestinal disorders such as chronic or acute diarrhea, malabsorption, abdominal pain, and inflammatory bowel diseases can indicate immune deficiency. The gastrointestinal tract is the largest lymphoid organ in the body, so it is not surprising that intestinal diseases are common among immunodeficient patients. Gastroenterologists therefore must be able to diagnose and treat patients with primary immunodeficiency. Immune-related gastrointestinal diseases can be classified as those that develop primarily via autoimmunity, infection, an inflammatory response, or malignancy. Immunodeficient and immunocompetent patients with gastrointestinal diseases present with similar symptoms. However, intestinal biopsy specimens from immunodeficient patients often have distinct histologic features, and these patients often fail to respond to conventional therapies. Therefore, early recognition of symptoms and referral to an immunologist for a basic immune evaluation is required to select appropriate treatments. Therapies for primary immunodeficiency comprise immunoglobulin replacement, antibiotics, and, in severe cases, bone marrow transplantation. Treatment of immunodeficient patients with concomitant gastrointestinal disease can be challenging, and therapy with immunomodulators often is required for severe disease. This review aims to guide gastroenterologists in the diagnosis and treatment of patients with primary immunodeficiency.

Idiopathic Non-Cirrhotic Intrahepatic Portal Hypertension (NCIPH)—Newer Insights into Pathogenesis and Emerging Newer Treatment Options

“…35,36 Earlier studies emphasized the importance of common variable immunodeficiency (CVID). 37,38 Treatment with intravenous immunoglobulin replacement has largely resolved the issue, further supporting the notion that enteropathies associated with CVID were somehow promoting the vasculopathy of terminal portal venous radicles. Such a sequence points strongly to a gastrointestinal source for the initiating factors in NCIPH.…”

IgA deficiency: a risk factor for food allergy-related atopic dermatitis in infants and young children

“…Therefore, defects in mounting a sufficient response against penetrating pathogens and failure to support the commensal organisms in the gastrointestinal tract in immunodeficient children predispose them to the breakdown of oral tolerance and to the subsequent development of food allergy, inflammatory bowel disease and infections [11]. Furthermore, the gastrointestinal tract is the largest immunological organ in the human body, rich in antibody-secreting plasma cells and an essential source of mucosal and circulating IgA and, to a lesser extent, also IgM and IgG isotypes playing a prominent role in mucosal compartmentalization of food antigens and protecting against allergic sensitization to foods [12][13][14]. Systemic sensitization to foods may thus reflect a lack of appropriate, antibody-dependent compartmentalization of food antigens.…”

A glance on recent progresses in diagnosis and treatment of primary immunodeficiencies/ Progrese recente în diagnosticul şi tratamentul imunodeficienţelor primare

“…However, it has to be stressed that for taking the correct actions the widely promulgated "warning signs" have to be interpreted by experts (21). Other tools to raise awareness might be medical discipline-specific reviews (22)(23)(24)(25)(26) or data mining in national or international registries. National registries may not have the power of international registries (http:// esid.org/Working-Parties/Registry/ESID-Online-Registry, accessed April 2014).…”