Common Variable Immune Deficiency
CVID involves the following:
(1) low levels of most or all of the immunoglobulin classes
(2) a lack of B lymphocytes or plasma cells capable of producing antibodies
(3) frequent bacterial infections.
The basic pathophysiologic process in CVID is a simple failure in the differentiation of B lymphocyte
Decreased serum levels of IgG and IgA are characteristic, 50% of patients also have diminished serum IgM levels and T-lymphocyte dysfunction.
Five distinct clinical phenotypes have been delineated for common variable immunodeficiency (CVID):
2.Autoimmunity – 20% pt’s develop an autoimmune disease.
3.Polyclonal lymphocytic infiltration
4.Enteropathic lymphoid malignancy
Treatment mainstay is Ig replacement therapy – stops cycle of infections.
Antimicrobial therapy should be initiated at the first sign of infection. The prophylactic use of antibiotics should be avoided because of an increased risk of infection with fungi or other resistant organisms.