Abstract
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.
Highlights
Autoimmune diseases of the gastrointestinal (GI) tract are increasingly growing worldwide over the last decades
The increasing body of evidence that primary immunodeficiency (PID) can complicate diagnostics of celiac disease (CeD) [3] and mimic inflammatory bowel disease (IBD) [6] implicates the need for a comprehensive review of this topic, especially when several studies have shown that autoimmune manifestations are the second most common manifestation of primary immunodeficiencies (PIDs) after infections [7,8]
Some researchers suggest that autoimmune disorders are developed in a course of PIDs as patients get older and, for this reason, autoimmunities are more common in adults than in children [9], making this topic even more relevant in terms of CeD and IBD
Summary
Autoimmune diseases of the gastrointestinal (GI) tract are increasingly growing worldwide over the last decades They concern both inflammatory bowel disease (IBD) and celiac disease (CeD) [1,2]. The increasing body of evidence that primary immunodeficiency (PID) can complicate diagnostics of CeD [3] and mimic IBD [6] implicates the need for a comprehensive review of this topic, especially when several studies have shown that autoimmune manifestations are the second most common manifestation of PIDs after infections [7,8]. This review aims to present the up-to-date knowledge on the incidence, pathophysiology, symptoms, diagnostics, and management of autoimmune GI diseases, CeD and IBD, in patients with underlying SIgAD or CVID. This review is focused on differences between the classic forms of the above-mentioned diseases and those observed in patients with compromised humoral immunity (as shown in Figure 1), to estimate whether we are facing a spectrum of one disease or different diseases characterized by a similar clinical manifestation
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
