Tehran University of Medical Sciences Immunology and Genetics Journal 2645-4831 3 2 2020 08 01 12 28 Mojde Soltani Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Mahnaz Rezaei Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran Mazdak Ganjalikhani Hakemi Department of Immunology, School of Medicine, Isfahan University of Medical Sciences, Isfahan, Iran 2020 04 15 Autoimmunity is observed by almost one-third of patients with CVID. Different mechanisms including genetic defects and dysregulation of innate and adaptive immunity leads to autoimmunity in these patients CVID. The clinical phenotypes of autoimmunity in CVID patients comprise fall in a wide spectrum, from organ-specific autoimmunity to systemic complications. The most common autoimmunity is autoimmune cytopenia in CVID patients. In this article, we have provided a collection of the most significant and recent information about prevalence, genetics, pathogenesis and clinical manifestations of autoimmunity in CVID patients, and provided an overview on its management and future perspective. https://igj.tums.ac.ir/index.php/igj/article/view/47 https://igj.tums.ac.ir/index.php/igj/article/download/47/37