Abstract
BackgroundPatients with primary immunodeficiency (PID) often suffer from frequent respiratory tract infections. Despite standard treatment with IgG-substitution and antibiotics many patients do not improve significantly. Therefore, we hypothesized that additional immune deficits may be present among these patients.ObjectiveTo investigate if PID patients exhibit impaired production of antimicrobial peptides (AMPs) in nasal fluid and a possible link between AMP-expression and Th17-cells.MethodsNasal fluid, nasopharyngeal swabs and peripheral blood mononuclear cells (PBMCs) were collected from patients and healthy controls. AMP levels were measured in nasal fluid by Western blotting. Nasal swabs were cultured for bacteria. PBMCs were stimulated with antigen and the supernatants were assessed for IL-17A release by ELISA.ResultsIn healthy controls and most patients, AMP levels in nasal fluid were increased in response to pathogenic bacteria. However, this increase was absent in patients with common variable immunodeficiency (CVID) and Hyper-IgE syndrome (HIES), despite the presence of pathogenic bacteria. Furthermore, stimulation of PBMCs revealed that both HIES and CVID patients exhibited an impaired production of IL-17A.ConclusionCVID and HIES patients appear to have a dysregulated AMP response to pathogenic bacteria in the upper respiratory tract, which could be linked to an aberrant Th17 cell response.
Highlights
Diseases that are characterized by lack of antibodies include syndromes such as selective IgAdeficiency, IgG-deficiency and common variable immunodeficiency (CVID)
This increase was absent in patients with common variable immunodeficiency (CVID) and Hyper-IgE syndrome (HIES), despite the presence of pathogenic bacteria
Stimulation of peripheral blood mononuclear cells (PBMCs) revealed that both HIES and CVID patients exhibited an impaired production of IL-17A
Summary
Diseases that are characterized by lack of antibodies (hypogammaglobulinemias, HGGs) include syndromes such as selective IgAdeficiency, IgG-deficiency and common variable immunodeficiency (CVID). Common symptoms include frequent bacterial respiratory tract infections (RTIs), fatigue as well as autoimmune manifestations [1]. Standard treatment involves antibiotic therapy and patients with low levels of IgG and/or IgA are administered IgG preparations, which in most cases reduce symptoms and improve overall health [6]. It is possible that immune deficits other than lack of immunoglobulins may be involved in some of these disorders. Such immune deficits could include two key components of the innate and adaptive immune system, namely antimicrobial peptides (AMPs) and Th17 cells. Patients with primary immunodeficiency (PID) often suffer from frequent respiratory tract infections. We hypothesized that additional immune deficits may be present among these patients
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