Gastrointestinal Disorders Associated with Primary Immunodeficiency Diseases

Clinical Reviews in Allergy & Immunology
Stella HartonoNicholas L Rider

Abstract

There are now 354 inborn errors of immunity (primary immunodeficiency diseases (PIDDs)) with 344 distinct molecular etiologies reported according to the International Union of Immunological Sciences (IUIS) (Clin Gastroenterol Hepatol 11: p. 1050-63, 2013, Semin Gastrointest Dis 8: p. 22-32, 1997, J Clin Immunol 38: p. 96-128, 2018). Using the IUIS document as a reference and cross-checking PubMed ( www.ncbi.nlm.nih.pubmed.gov ), we found that approximately one third of the 354 diseases of impaired immunity have a gastrointestinal component [J Clin Immunol 38: p. 96-128, 2018]. Often, the gastrointestinal symptomatology and pathology is the heralding sign of a PIDD; therefore, it is important to recognize patterns of disease which may manifest along the gastrointestinal tract as a more global derangement of immune function. As such, holistic consideration of immunity is warranted in patients with clinically significant gastrointestinal disease. Here, we discuss the manifold presentations and GI-specific complications of PIDDs which could lead patients to seek advice from a variety of clinician specialists. Often, patients with these medical problems will engage general pediatricians, surgeons, gastroenterologists, rheumatologist...Continue Reading

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Citations

Feb 28, 2020·Nature·Daniel B Graham, Ramnik J Xavier
Sep 9, 2020·Scandinavian Journal of Immunology·Dharmagat BhattaraiAmit Rawat
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Nov 19, 2020·Journal of Clinical Immunology·Grant PickettNicholas L Rider
Nov 12, 2020·Jornal de pediatria·Fernanda Pinto-Mariz

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