Autoimmune manifestations in common variable immunodeficiency

Common variable immunodeficiency (CVID) is a disorder characterized by decreased serum immunoglobulin concentrations and increased incidence of recurrent infections. Interestingly 20-25% of patients with CVID develop clinical features suggestive of an autoimmune disease. Although this association is well established, the immunodeficiency background of CVID patients manifesting autoimmune disorders is often overlooked. This study describes three CVID patients displaying a variety of autoimmune manifestations. The pathophysiologic mechanisms of autoimmunity in CVID are also reviewed.