title: Delay in diagnosis affects the clinical outcome in a cohort of cvid patients with marked reduction of iga serum levels.
abstract: Common variable immunodeficiency disorders (CVID) represent a collection of diseases leading to an absent or strongly impaired antibody production. CVID presents a wide range of immunological abnormalities and clinical manifestations, including infections, inflammatory and autoimmune diseases, and malignancies. The aim of this observational study was to analyze the epidemiological and clinical features of a cohort of 75 Italian CVID patients, and evaluate the correlation with comorbidity and mortality. Clinical data were retrospectively collected: the cohort was followed-up for a maximum of 30years (mean time of 10.24years, median of 9years). An higher age at the diagnosis of CVID and an higher age at onset of symptoms were significantly associated with a reduction of patients survival if stratified per median of IgA (less than or >8.00mg/dl). Thus IgA levels at diagnosis are correlated with patients survival contributing to identify a subset with a worse prognostic outcome.
authors.affiliation: Department of Advanced Biomedical Sciences, Azienda Ospedaliera Universitaria, University of Naples Federico II, Naples, Italy.
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Graziano
authors.affiliation: Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Pecoraro
authors.affiliation: Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Mormile
authors.affiliation: Department of Advanced Biomedical Sciences, Azienda Ospedaliera Universitaria, University of Naples Federico II, Naples, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Quaremba
authors.affiliation: Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy. Electronic address: argenove@unina.it.
authors.forename: Arturo
authors.initials: A
authors.lastname: Genovese
authors.affiliation: Department of Advanced Biomedical Sciences, Azienda Ospedaliera Universitaria, University of Naples Federico II, Naples, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Buccelli
authors.affiliation: Department of Advanced Biomedical Sciences, Azienda Ospedaliera Universitaria, University of Naples Federico II, Naples, Italy.
authors.forename: Mariano
authors.initials: M
authors.lastname: Paternoster
authors.affiliation: Department of Translational Medical Sciences, Allergy and Clinical Immunology, University of Naples Federico II, Naples, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Spadaro
mesh_terms: D000328:Adult; D000368:Aged; D015331:Cohort Studies; D017074:Common Variable Immunodeficiency / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged
chemical_list: D007070:Immunoglobulin A
keywords: Common variable immunodeficiency; Diagnostic delay; Hypogammaglobulinemia; IgA deficiency; Primary antibody deficiency