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Common Variable Immunodeficiency: Epidemiology, Pathogenesis, Clinical Manifestations, Diagnosis, Classification, and Management

Yazdani R1, Habibi S1, Sharifi L2, Azizi G3, Abolhassani H4,5, Olbrich P6, Aghamohammadi A1

1Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children's Medical Center, Tehran University of Medical Science, Tehran, Iran.
2Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
3Non-Communicable Diseases Research Center, Alborz University of Medical Sciences, Karaj, Iran.
4Research Center for Primary Immunodeficiencies, Iran University of Medical Science, Tehran, Iran.
5Division of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institute at Karolinska University Hospital Huddinge, Stockholm, Sweden.
6Sección de Infectología e Inmunopatología, Unidad de Pediatría, Hospital Virgen del Rocío/Instituto de Biomedicina de Sevilla (IBiS), Seville, Spain.

J Investig Allergol Clin Immunol 2020; Vol 30(1) : 14-34
doi: 10.18176/jiaci.0388

Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by hypogammaglobulinemia and increased susceptibility to recurrent bacterial infections. It is the most frequent symptomatic antibody deficiency, with a wide variety of infectious and noninfectious complications. Numerous studies have demonstrated that immunological and genetic defects are involved in the pathogenesis of CVID. However, in most cases, the genetic background of the disease remains unidentified. This review aims to discuss various aspects of CVID, including epidemiology, pathogenesis, symptoms, diagnosis, classification, and management.

Key words: Common variable immunodeficiency, Epidemiology, Pathogenesis, Symptoms, Diagnosis, Classifications, Management