Research Center for Immunodeficiencies, Pediatrics Center of Excellence, Children´s Medical Center, Tehran University of Medical Science, Tehran, Iran.
Uro-Oncology Research Center, Tehran University of Medical Sciences, Tehran, Iran.
J Investig Allergol Clin Immunol. 2020;30(1):14-34. doi: 10.18176/jiaci.0388. Epub 2019 Feb 11.
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.
普通变异性免疫缺陷症(CVID)是一种以低丙种球蛋白血症和反复发生细菌性感染为特征的异质性疾病。它是最常见的有症状抗体缺陷症,伴有多种感染性和非感染性并发症。大量研究表明,免疫和遗传缺陷与 CVID 的发病机制有关。然而,在大多数情况下,该疾病的遗传背景仍未确定。本综述旨在讨论 CVID 的各个方面,包括流行病学、发病机制、症状、诊断、分类和管理。