title: Chronic lung disease in common variable immune deficiency (CVID): A pathophysiological role for microbial and non-B cell immune factors.
abstract: One of the most common and most severe forms of primary antibody deficiency encountered in the clinical setting is a heterogeneous group of syndromes termed common variable immune deficiency (CVID). This disorder is characterized by reduced immunoglobulin production and increased susceptibility to infection, particularly of the respiratory tract. Infection and subsequent immunological/inflammatory processes may contribute to the development of pulmonary complications such as bronchiectasis and interstitial lung disease. Immunoglobulin replacement and/or antibiotic therapy, to prevent infection, are routinely prescribed treatments. However, chronic lung disease, the major cause of morbidity and mortality in this patient cohort, may still progress. This clinical progression suggests that pathogens recalcitrant to currently prescribed treatments and other immunological defects may be contributing to the development of pulmonary disease. This review describes the potential role of microbiological and non-B cell immunological factors, including T-cells, neutrophils, complement, toll like receptors, and antimicrobial peptides, in the pathogenicity of chronic lung disease in patients with CVID.
authors.affiliation: a Halo Research Group, Queen's University Belfast , Belfast , United Kingdom.|b Centre for Experimental Medicine, School of Medicine , Dentistry and Biomedical Sciences. Queen's University Belfast , Belfast , United Kingdom.
authors.forename: Denver
authors.initials: D
authors.lastname: Mooney
authors.affiliation: c T he Royal Hospitals, Belfast Health and Social Care Trust , Regional Immunology Service , Belfast , United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Edgar
authors.affiliation: a Halo Research Group, Queen's University Belfast , Belfast , United Kingdom.|b Centre for Experimental Medicine, School of Medicine , Dentistry and Biomedical Sciences. Queen's University Belfast , Belfast , United Kingdom.
authors.forename: Gisli
authors.initials: G
authors.lastname: Einarsson
authors.affiliation: d Belfast City Hospital, Belfast Health and Social Care Trust , Regional Respiratory Centre , Belfast , United Kingdom.
authors.forename: Damian
authors.initials: D
authors.lastname: Downey
authors.affiliation: a Halo Research Group, Queen's University Belfast , Belfast , United Kingdom.|b Centre for Experimental Medicine, School of Medicine , Dentistry and Biomedical Sciences. Queen's University Belfast , Belfast , United Kingdom.
authors.forename: Stuart
authors.initials: S
authors.lastname: Elborn
authors.affiliation: a Halo Research Group, Queen's University Belfast , Belfast , United Kingdom.|e School of Pharmacy , Queen's University Belfast , Belfast , United Kingdom.
authors.forename: Michael
authors.initials: M
authors.lastname: Tunney
mesh_terms: D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D001424:Bacterial Infections / Q000276:immunology / Q000473:pathology*; D001987:Bronchiectasis / Q000276:immunology / Q000473:pathology*; D002908:Chronic Disease; D017074:Common Variable Immunodeficiency / Q000175:diagnosis / Q000473:pathology*; D003165:Complement System Proteins / Q000276:immunology; D018450:Disease Progression; D006801:Humans; D007155:Immunologic Factors / Q000276:immunology; D017563:Lung Diseases, Interstitial / Q000276:immunology / Q000473:pathology*; D009504:Neutrophils / Q000276:immunology; D012141:Respiratory Tract Infections / Q000276:immunology / Q000382:microbiology / Q000473:pathology*; D013601:T-Lymphocytes / Q000276:immunology; D014777:Virus Diseases / Q000276:immunology / Q000473:pathology*
chemical_list: D000900:Anti-Bacterial Agents; D007155:Immunologic Factors; D003165:Complement System Proteins
keywords: Primary antibody deficiency; adaptive and innate immune deficiencies; bronchiectasis; respiratory tract infection; treatment and disease progression