Hypogammaglobulinemia and Common Variable Immunodeficiency

Common variable immune deficiency (CVID) is a heterogeneous group of primary immunodeficiencies characterized by hypogammaglobulinemia and poor to absent specific antibody responses to vaccination. Due to prevalence and numbers of medical encounters, the awareness of CVID is highly relevant to pediatricians, internists, and primary care physicians, as well as pulmonologists, otolaryngologists and physicians in many other specialties. The incidence of CVID is estimated at between 1:20,000 and 1:50,000 in Caucasians, but there are regional differences, with CVID being less commonly diagnosed in Asians and Afro-Americans. The onset of symptoms may be at any age, but is generally between the ages of 15 and 40 years. Partially due to the somewhat later onset, the often more subtle presentation, and heterogeneous clinical appearance, a diagnostic delay of 5 to 8 years after the first cardinal symptom is common in all countries investigated, and has not changed in the past few years.