Common variable immunodeficiency (CVID) is the most common primary immune deficiency characterized by impaired production of specific immunoglobulin. The clinical manifestations are heterogeneous including acquisition of recurrent bacterial infections after a period of wellness, lymphoproliferation, autoimmunity, pulmonary disease, liver disease, enteropathy, granulomas, and an increased risk of malignancy. The etiology of CVID is largely unknown, with a considerable number of patients having an underlying genetic defect causing immune dysregulation. The antibody deficiency found in CVID is treated with lifelong immunoglobulin therapy, which is preventative of the majority of infections when given regularly.
Keywords: Autoimmunity; Common variable immunodeficiency; Hypogammaglobulinemia; Primary immune deficiency.
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