title: Important Factors Influencing Severity of Common Variable Immunodeficiency.
abstract: BACKGROUND
  Common variable immunodeficiency (CVID) is a primary immune deficiency with heterogeneous complications. The purpose of this study is to determine disease severity in a cohort of CVID patients based on the suggested scoring system and investigate predisposing factors which would be helpful to predict the severity of the disease.
  
  
  METHODS
  The study population comprised 113 CVID patients (69 males and 44 females) who were visited at Children's Medical Center (Pediatrics Center of Excellence affiliated to Tehran University of Medical Sciences, Tehran, Iran) during the last 30 years (from 1984-2014). According to a suggested severity scoring system, patients were divided into two groups, A and B. The clinical severity of the disease in patients was assessed with severity scores including 15 unlucky complications of the disease such as numbers of past meningitis, encephalitis or pneumonias, development of bronchopulmonary pathologies, presence of lymphoproliferative disorders, autoimmunity or malignancy.
  
  
  RESULTS
  The mean serum IgG level was significantly higher in group B (308.6±195.9) compared to group A (177.8 ± 151.9; P = 0.03). Patients in group B had a significantly higher percentage of CD8 (P = 0.003). However, they had lower percentage of CD4 lymphocytes (P = 0.08), switched memory B cells (CD27+IgM-IgD-) (P < 0.01) and regulatory T cells (P = 0.02) than group A.
  
  
  CONCLUSIONS
  Using standard and universal scoring system and understanding of related factors can be applicable in clinical settings for prognosis assessment of CVID patients.
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Mahisa
authors.initials: M
authors.lastname: Mokhtari
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Alireza
authors.initials: A
authors.lastname: Shakeri
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Babak
authors.initials: B
authors.lastname: Mirminachi
authors.affiliation: 1)Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 2)Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital Huddinge, Stockholm, Sweden.
authors.forename: Hassan
authors.initials: H
authors.lastname: Abolhassani
authors.affiliation: Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran.
authors.forename: Reza
authors.initials: R
authors.lastname: Yazdani
authors.affiliation: Center for Chronic Immunodeficiency, University Medical Center Freiburg and University of Freiburg, Germany.
authors.forename: Bodo
authors.initials: B
authors.lastname: Grimbacher
authors.affiliation: Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Asghar
authors.initials: A
authors.lastname: Aghamohammadi
mesh_terms: D000293:Adolescent; D000328:Adult; D015551:Autoimmunity; D016516:CD4-CD8 Ratio; D002648:Child; D002675:Child, Preschool; D017074:Common Variable Immunodeficiency / Q000097:blood* / Q000401:mortality*; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007223:Infant; D007492:Iran; D016014:Linear Models; D008297:Male; D011788:Quality of Life; D012189:Retrospective Studies; D012720:Severity of Illness Index; D015996:Survival Rate; D055815:Young Adult
chemical_list: D007074:Immunoglobulin G