Good’s Syndrome, CVID, and Selective Antibody Deficiency in Patients with Chronic Rhinosinusitis

Abstract

Good's syndrome and common variable immune deficiency (CVID) are associated with chronic rhinosinusitis. Good's syndrome is characterized by hypogammaglobulinemia, B-cell depletion, variable defects in cellular immunity and thymoma. Immunodeficiency and recurrent infections can initially present after thymectomy. The pathogenesis can involve cytokines from bone marrow along with genetic defects. Intravenous gamma globulin (IVIG) restores defective signaling and can reestablish immune homeostasis. IVIG at regular intervals is the most effective way to improve the clinical symptoms and reduce patient mortality. Increased awareness of the clinical and immunological profile of this syndrome may increase its early recognition. CVID patients have hypogammaglobulinemia, respond to IVIG and have a dysregulated antimicrobial peptide response to pathogenic bacteria in the upper respiratory tract. This article reviewed selected literature on Good's syndrome, described an unusual case of Good's syndrome, CVID including SAD related to chronic rhinosinusitis.