title: Comparisons of CVID and IgGSD: referring physicians, autoimmune conditions, pneumovax reactivity, immunoglobulin levels, blood lymphocyte subsets, and HLA-A and -B typing in 432 adult index patients.
abstract: Common variable immunodeficiency (CVID) and immunoglobulin (Ig) G subclass deficiency (IgGSD) are heterogeneous disorders characterized by respiratory tract infections, selective Ig isotype deficiencies, and impaired antibody responses to polysaccharide antigens. Using univariable analyses, we compared observations in 34 CVID and 398 IgGSD adult index patients (81.9% women) referred to a hematology/oncology practice. Similarities included specialties of referring physicians, mean ages, proportions of women, reactivity to Pneumovax, median serum IgG3 and IgG4 levels, median blood CD56+/CD16+ lymphocyte levels, positivity for HLA-A and -B types, and frequencies of selected HLA-A, -B haplotypes. Dissimilarities included greater prevalence of autoimmune conditions, lower median IgG, IgA, and IgM, and lower median CD19+, CD3+/CD4+, and CD3+/CD8+ blood lymphocytes in CVID patients. Prevalence of Sjögren's syndrome and hypothyroidism was significantly greater in CVID patients. Combined subnormal IgG1/IgG3 occurred in 59% and 29% of CVID and IgGSD patients, respectively. Isolated subnormal IgG3 occurred in 121 IgGSD patients (88% women). Logistic regression on CVID (versus IgGSD) revealed a significant positive association with autoimmune conditions and significant negative associations with IgG1, IgG3, and IgA and CD56+/CD16+ lymphocyte levels, but the odds ratio was increased for autoimmune conditions alone (6.9 (95% CI 1.3, 35.5)).
authors.affiliation: Department of Medicine, Brookwood Medical Center, Suite 626, 2022 Brookwood Medical Center Drive, Birmingham, AL 35209, USA ; Southern Iron Disorders Center, Birmingham, AL 35209, USA ; Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA.
authors.forename: James C
authors.initials: JC
authors.lastname: Barton
authors.affiliation: Department of Medicine, Brookwood Medical Center, Suite 626, 2022 Brookwood Medical Center Drive, Birmingham, AL 35209, USA ; Southern Iron Disorders Center, Birmingham, AL 35209, USA ; Brookwood Biomedical, Birmingham, AL 35209, USA.
authors.forename: Luigi F
authors.initials: LF
authors.lastname: Bertoli
authors.affiliation: Southern Iron Disorders Center, Birmingham, AL 35209, USA.
authors.forename: J Clayborn
authors.initials: JC
authors.lastname: Barton
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000276:immunology; D017252:CD3 Complex / Q000276:immunology / Q000378:metabolism; D019002:CD56 Antigen / Q000276:immunology / Q000378:metabolism; D017074:Common Variable Immunodeficiency / Q000097:blood / Q000235:genetics* / Q000276:immunology*; D005260:Female; D015234:HLA-A Antigens / Q000235:genetics / Q000276:immunology; D015235:HLA-B Antigens / Q000235:genetics / Q000276:immunology; D006239:Haplotypes / Q000235:genetics / Q000276:immunology; D006801:Humans; D017099:IgG Deficiency / Q000097:blood / Q000235:genetics* / Q000276:immunology*; D007070:Immunoglobulin A / Q000097:blood / Q000276:immunology; D007074:Immunoglobulin G / Q000097:blood / Q000276:immunology; D007075:Immunoglobulin M / Q000097:blood / Q000276:immunology; D016015:Logistic Models; D016131:Lymphocyte Subsets / Q000276:immunology / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D022242:Pneumococcal Vaccines / Q000276:immunology; D017452:Receptors, IgG / Q000276:immunology / Q000378:metabolism; D012017:Referral and Consultation; D013296:Streptococcus pneumoniae / Q000276:immunology
chemical_list: D017252:CD3 Complex; D019002:CD56 Antigen; D015234:HLA-A Antigens; D015235:HLA-B Antigens; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D022242:Pneumococcal Vaccines; D017452:Receptors, IgG