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Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults

1
Department of Gastroenterology, Dietetics and Internal Diseases, Poznan University of Medical Sciences, 60-355 Poznan, Poland
2
Institute of Human Genetics, Polish Academy of Sciences Poznan, 60-479 Poznan, Poland
3
Department of Pharmaceutical Technology, Faculty of Pharmacy, University of Coimbra, Pólo das Ciências da Saúde, Azinhaga de Santa Comba, 3000-548 Coimbra, Portugal
4
CEB—Centre of Biological Engineering, University of Minho, Campus de Gualtar, 4710-057 Braga, Portugal
*
Author to whom correspondence should be addressed.
These authors contributed equally to this work: Ida Judyta Malesza and Michał Malesza.
Int. J. Mol. Sci. 2020, 21(15), 5223; https://doi.org/10.3390/ijms21155223
Received: 30 June 2020 / Revised: 20 July 2020 / Accepted: 21 July 2020 / Published: 23 July 2020
(This article belongs to the Special Issue Immunoglobulins in Inflammation)
In recent years, the incidence of immune-mediated gastrointestinal disorders, including celiac disease (CeD) and inflammatory bowel disease (IBD), is increasingly growing worldwide. This generates a need to elucidate the conditions that may compromise the diagnosis and treatment of such gastrointestinal disorders. It is well established that primary immunodeficiencies (PIDs) exhibit gastrointestinal manifestations and mimic other diseases, including CeD and IBD. PIDs are often considered pediatric ailments, whereas between 25 and 45% of PIDs are diagnosed in adults. The most common PIDs in adults are the selective immunoglobulin A deficiency (SIgAD) and the common variable immunodeficiency (CVID). A trend to autoimmunity occurs, while gastrointestinal disorders are common in both diseases. Besides, the occurrence of CeD and IBD in SIgAD/CVID patients is significantly higher than in the general population. However, some differences concerning diagnostics and management between enteropathy/colitis in PIDs, as compared to idiopathic forms of CeD/IBD, have been described. There is an ongoing discussion whether CeD and IBD in CVID patients should be considered a true CeD and IBD or just CeD-like and IBD-like diseases. This review addresses the current state of the art of the most common primary immunodeficiencies in adults and co-occurring CeD and IBD. View Full-Text
Keywords: primary immunodeficiency; selective IgA deficiency; common variable immunodeficiency; celiac disease; inflammatory bowel disease; Crohn’s disease; ulcerative colitis primary immunodeficiency; selective IgA deficiency; common variable immunodeficiency; celiac disease; inflammatory bowel disease; Crohn’s disease; ulcerative colitis
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Malesza, I.J.; Malesza, M.; Krela-Kaźmierczak, I.; Zielińska, A.; Souto, E.B.; Dobrowolska, A.; Eder, P. Primary Humoral Immune Deficiencies: Overlooked Mimickers of Chronic Immune-Mediated Gastrointestinal Diseases in Adults. Int. J. Mol. Sci. 2020, 21, 5223.

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