title: Common variable immunodeficiency in systemic lupus erythematosus.
abstract: OBJECTIVE
  The development of common variable immunodeficiency (CVID) or hypogammaglobulinemia in systemic lupus erythematosus (SLE) is rare. The purpose of this article is to provide a detailed review of lupus-associated CVID and to identify clinical characteristics and laboratory features in patients with SLE-associated CVID.
  
  
  METHODS
  We describe 2 patients with SLE and CVID and review the cases published in the English literature highlighting both the demographic and the clinical characteristics and the laboratory and therapeutic aspects of this disorder.
  
  
  RESULTS
  Detailed descriptions of 18 patients were available; 89% were females with a mean age at the onset of SLE of 23.8 years. In 50% of patients CVID developed within the first 5 years after the diagnosis of SLE. All patients had been treated with corticosteroids and 72% had also received immunosuppressive therapy. Sinopulmonary infections were the most frequent symptom. SLE disease activity decreased after the development of CVID in 67% of patients. Most patients (89%) were treated with gammaglobulin therapy. The most notable immunological feature was a reduced number or percentage of B-cells in 60% of patients.
  
  
  CONCLUSIONS
  CVID should be suspected in any SLE patient with recurrent sinopulmonary infections in the absence of SLE activity and/or immunosuppressive treatment.
authors.affiliation: Department of Rheumatology, Hospital Universitario Puerta de Hierro, Universidad Autónoma de Madrid, Madrid, Spain.
authors.forename: Mónica
authors.initials: M
authors.lastname: Fernández-Castro
authors.forename: Susana
authors.initials: S
authors.lastname: Mellor-Pita
authors.forename: María Jesús
authors.initials: MJ
authors.lastname: Citores
authors.forename: Pilar
authors.initials: P
authors.lastname: Muñoz
authors.forename: Pablo
authors.initials: P
authors.lastname: Tutor-Ureta
authors.forename: Lucia
authors.initials: L
authors.lastname: Silva
authors.forename: Juan Antonio
authors.initials: JA
authors.lastname: Vargas
authors.forename: Miguel
authors.initials: M
authors.lastname: Yebra-Bango
authors.forename: José Luis
authors.initials: JL
authors.lastname: Andreu
mesh_terms: D000293:Adolescent; D000328:Adult; D017074:Common Variable Immunodeficiency / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000276:immunology; D016131:Lymphocyte Subsets / Q000145:classification*
chemical_list: D016756:Immunoglobulins, Intravenous