What is the role of IVIG in the treatment of common variable immunodeficiency (CVID)?

Updated: Oct 16, 2018
  • Author: C Lucy Park, MD; Chief Editor: Harumi Jyonouchi, MD  more...
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Answer

Immunoglobulin (Ig) replacement therapy, by intravenous infusion or subcutaneous injection, remains the mainstay of therapy. The primary goal is the prevention of infection. Ig replacement therapy has decreased the frequency of life-threatening infections in these patients significantly. Most patients with common variable immunodeficiency (CVID) and sinopulmonary disease without severe bronchiectasis do well once they are placed on regular intravenous immunoglobulin therapy. If replacement therapy is started early, and if appropriate amounts are given with sufficient frequency, the cycle of recurrent infections and progressive lung damage can be arrested. However, silent progression of bronchiectasis was reported in a small number of patients while receiving adequate Ig replacement therapy.

Currently available immunoglobulin products in the United States are derived from pooled human plasma (see Medication). The manufacturing processes include cold ethanol fractionation of Ig and viral inactivation and removal steps. Biological activity of the IgG molecule, not simply the antibody titer, but opsonic and complement activity and circulating half-life, may be affected by discrete steps in the manufacturing and isolation of IgG. Only one report has compared two different IVIG products. In this randomized double-blind multicenter study, the Gamunex (purified using caprylate treatment and chromatography) treated group had a significantly lower number of infections compared with the group treated with Gamimune N (solvent-detergent treated) from the same company (annual infection rates were 0.18 compared with 0.43; p=0.023).

Reportedly, IVIG significantly decreased the frequency of lower respiratory tract and severe infection; however, IVIG did not change the frequency of nonrespiratory or upper respiratory infections. Also IVIG did not change the clinical course of autoimmune manifestations in patients with common variable immunodeficiency.


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