title: Altered fraction of regulatory B and T cells is correlated with autoimmune phenomena and splenomegaly in patients with CVID.
abstract: Common variable immunodeficiency (CVID) is a heterogeneous primary immunodeficiency disease, leading to recurrent bacterial airway infections and often also autoimmune complications. To shed light on the regulatory lymphocytes from these patients, we analyzed the levels of regulatory B (pro-B10) cell and regulatory T (Treg) cell subpopulations in PBMCs from twenty-six patients diagnosed with CVID using multi-color flowcytometry. Pro-B10 cells were induced by 48h in vitro stimulation prior to analysis. Suppressor function was measured on a subset of patients with splenomegaly and autoimmune complications. The levels of regulatory B and T cells were correlated to clinical manifestations, including autoimmunity, splenomegaly and CVID EUROclass subgroups. We demonstrate a significant association between elevated levels of pro-B10 cells, decreased levels of Tregs and autoimmune phenomena in CVID patients. The finding of marked abnormalities in regulatory lymphocyte populations contribute to our understanding of the pathogenesis of CVID and potentially be valuable in the clinical management and treatment of patients.
authors.affiliation: International Center for Immunodeficiency Diseases, Aarhus University Hospital Skejby, Aarhus, Denmark; Department of Health Science and Technology, Aalborg University, Aalborg, Denmark; Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark. Electronic address: ekol@hst.aau.dk.
authors.forename: Emil
authors.initials: E
authors.lastname: Kofod-Olsen
authors.affiliation: Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark; Department of Biomedicine, Aarhus University, Aarhus, Denmark.
authors.forename: Sofie Eg
authors.initials: SE
authors.lastname: Jørgensen
authors.affiliation: Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark; Department of Biomedicine, Aarhus University, Aarhus, Denmark.
authors.forename: Sara Konstantin
authors.initials: SK
authors.lastname: Nissen
authors.affiliation: Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark.
authors.forename: Lena
authors.initials: L
authors.lastname: Westh
authors.affiliation: International Center for Immunodeficiency Diseases, Aarhus University Hospital Skejby, Aarhus, Denmark; Department of Clinical Immunology, Aarhus University Hospital, Skejby, Aarhus, Denmark.
authors.forename: Bjarne K
authors.initials: BK
authors.lastname: Møller
authors.affiliation: International Center for Immunodeficiency Diseases, Aarhus University Hospital Skejby, Aarhus, Denmark; Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark.
authors.forename: Lars
authors.initials: L
authors.lastname: Østergaard
authors.affiliation: International Center for Immunodeficiency Diseases, Aarhus University Hospital Skejby, Aarhus, Denmark; Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark.
authors.forename: Carsten S
authors.initials: CS
authors.lastname: Larsen
authors.affiliation: International Center for Immunodeficiency Diseases, Aarhus University Hospital Skejby, Aarhus, Denmark; Department of Infectious Diseases, Aarhus University Hospital, Skejby, Aarhus, Denmark; Department of Biomedicine, Aarhus University, Aarhus, Denmark.
authors.forename: Trine H
authors.initials: TH
authors.lastname: Mogensen
mesh_terms: D017668:Age of Onset; D015551:Autoimmunity / Q000276:immunology; D060151:B-Lymphocytes, Regulatory / Q000166:cytology / Q000276:immunology*; D017074:Common Variable Immunodeficiency / Q000276:immunology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D013163:Splenomegaly / Q000503:physiopathology*; D050378:T-Lymphocytes, Regulatory / Q000166:cytology / Q000276:immunology*
keywords: Autoimmunity; B10 cells; CVID; Common variable immunodeficiency; Regulatory B cells; Regulatory T cells; Treg; aTreg; rTreg