Mahisa Mokhtari1, Alireza Shakeri1, Babak Mirminachi1, Hassan Abolhassani2, Reza Yazdani3, Bodo Grimbacher4, Asghar Aghamohammadi1. 1. Research Center for Immunodeficiencies, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran. 2. 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. 3. Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran. 4. Center for Chronic Immunodeficiency, University Medical Center Freiburg and University of Freiburg, Germany.
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. CONCLUSION: Using standard and universal scoring system and understanding of related factors can be applicable in clinical settings for prognosis assessment of CVID patients.
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 CVIDpatients 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 CVIDpatients (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. CONCLUSION: Using standard and universal scoring system and understanding of related factors can be applicable in clinical settings for prognosis assessment of CVIDpatients.
Authors: Markus G Seidel; Victoria K Tesch; Linlin Yang; Fabian Hauck; Anna Lena Horn; Maria Anna Smolle; Franz Quehenberger; Martin Benesch Journal: J Clin Immunol Date: 2021-11-19 Impact factor: 8.542