title: Gastrointestinal Disorders Associated with Common Variable Immune Deficiency (CVID) and Chronic Granulomatous Disease (CGD).
abstract: Common variable immune deficiency (CVID) and chronic granulomatous disease (CGD) are two of the well-characterized primary immune deficiencies with distinct pathologic defects. While CVID is predominantly a disorder of the adaptive immune system, in CGD, innate immunity is impaired. In both syndromes, the clinical manifestations include an increased susceptibility to infections and a number of non-infectious, inflammatory conditions including systemic autoimmunity, as well as organ-specific pathology. Among the organ-associated disorders, gastrointestinal (GI) manifestations are one of the most intractable. As such, non-infectious inflammatory disorders of the GI tract are clinically challenging as they have protean manifestations, often resembling inflammatory bowel disease (IBD) or celiac disease, are notoriously difficult to treat, and hence are associated with significant morbidity and mortality. Therefore, assessing the pathogenesis and defining appropriate therapeutic approaches for GI disease in patients with CVID and CGD is imperative.
authors.affiliation: Division of Gastroenterology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.|The Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Uzzan
authors.affiliation: Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.
authors.forename: Huaibin M
authors.initials: HM
authors.lastname: Ko
authors.affiliation: Division of Gastroenterology, Department of Medicine, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.|The Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.
authors.forename: Saurabh
authors.initials: S
authors.lastname: Mehandru
authors.affiliation: The Immunology Institute, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA. charlotte.cunningham-rundles@mssm.edu.|Division of Allergy and Clinical Immunology, Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA. charlotte.cunningham-rundles@mssm.edu.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Cunningham-Rundles
mesh_terms: D017074:Common Variable Immunodeficiency / Q000150:complications*; D005767:Gastrointestinal Diseases / Q000188:drug therapy / Q000209:etiology*; D005770:Gastrointestinal Neoplasms / Q000209:etiology; D006105:Granulomatous Disease, Chronic / Q000150:complications*; D006801:Humans; D009894:Opportunistic Infections / Q000150:complications
keywords: Chronic enteropathy; Chronic granulomatous disease (CGD); Common variable immune deficiency (CVID); Gastrointestinal tract; Inflammatory bowel diseases; Primary immune deficiency; Villous atrophy