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- Title
Gastrointestinal Involvement in Primary Antibody Deficiencies.
- Authors
Milota, Tomas; Smetanova, Jitka; Klojdova, Iveta
- Abstract
Primary antibody deficiencies (PADs) are the most frequent group of inborn errors of immunity. Impaired B-cell development, reduced production of immunoglobulins (mainly IgG and IgA), and specific antibodies resulting in recurrent infections are their hallmarks. Infections typically affect the respiratory tract; however, gastrointestinal involvement is also common. These include infection with Helicobacter pylori, Salmonella, Campylobacter species, Giardia, and noroviruses. Impaired IgA production also contributes to dysbiosis and thereby an increase in abundance of species with proinflammatory properties, resulting in immune system dysregulation. Dysregulation of the immune system results in a broad spectrum of non-infectious manifestations, including autoimmune, lymphoproliferative, and granulomatous complications. Additionally, it increases the risk of malignancy, which may be present in more than half of patients with PADs. Higher prevalence is often seen in monogenic causes, and gastrointestinal involvement may clinically mimic various conditions including inflammatory bowel diseases and celiac disease but possess different immunological features and response to standard treatment, which make diagnosis and therapy challenging. The spectrum of malignancies includes gastric cancer and lymphoma. Thus, non-infectious manifestations significantly affect mortality and morbidity. In this overview, we provide a comprehensive insight into the epidemiology, genetic background, pathophysiology, and clinical manifestations of infectious and non-infectious complications.
- Subjects
CZECH Republic; BIOTHERAPY; LYMPHOMA risk factors; THERAPEUTIC use of monoclonal antibodies; PRIMARY immunodeficiency diseases; NON-communicable diseases; GIARDIASIS; STOMACH tumors; COMMUNICABLE diseases; SALMONELLA diseases; INFLAMMATORY bowel diseases; IMMUNOGLOBULINS; ADRENOCORTICAL hormones; GUT microbiome; GASTROINTESTINAL diseases; CAMPYLOBACTER infections; AUTOIMMUNE diseases; COMMON variable immunodeficiency; GENETIC testing; RISK assessment; CELIAC disease; NOROVIRUS diseases; AGAMMAGLOBULINEMIA; LYMPHOPROLIFERATIVE disorders; IMMUNOSUPPRESSIVE agents; HELICOBACTER diseases; DISEASE risk factors; DISEASE complications; SYMPTOMS
- Publication
Gastrointestinal Disorders, 2023, Vol 5, Issue 1, p52
- ISSN
2624-5647
- Publication type
Article
- DOI
10.3390/gidisord5010006