Efficacy of Intravenous Gammaglobulin for Immunoglobulin G Subclass and/or Antibody Deficiency in Adults

Abstract

Background: The clinical significance and efficacy of treating patients who have immunoglobulin (Ig) G subclass deficiency and/or antibody deficiency with Ig-replacement therapy has been debated. There are no clear guidelines to recommend intravenous gammaglobulin (IgIV) in these patients as there are few published studies documenting its efficacy. Methods: We studied in an open-label protocol 10 adult patients with recurrent respiratory infections and IgG subclass and/or antibody deficiency. All patients received monthly IgIV for 12 months and then were observed for 3 months without IgIV infusions. We studied quality of life, incidence of infections, need for antibiotics, frequency of hospitalizations due to infections, IgG subclass and antibody (tetanus and pneumococcal) levels. Innate immunity was evaluated by studying the status of Toll-like receptors and polymorphisms, mannan-binding lectin levels and genotypes. Correction of the immune defects during IgIV therapy was evaluated. Results: Monthly IgIV significantly improved quality of life, decreased the number of infections and the need for antibiotics, and improved IgG subclass and antibody serum levels. No consistent finding of innate immunity could be detected. Conclusions: IgIV could be beneficial in patients with IgG subclass or antibody deficiency.

Retinal Vasculitis Revealing Immunoglobulin G Subclass Deficiency

“…Systemic steroids are generally efficient on autoimmune retinal vasculitis, 1,8,9 and this was the case in our patient since hyalitis and vasculitis improved rapidly after the onset of oral prednisolone. Additionally, the onset of monthly perfusions of immunoglobulins resulted in an improvement of the general conditions and the lack of ocular relapses during a 2-year follow-up.…”

Immunoglobulin treatment in primary antibody deficiency

“…In an open-label study [67], 10 adult patients with symptomatic IgG subclass deficiency were treated with monthly IVIg for 1 year followed by 3 months of observation off IVIg therapy. All patients showed a significant reduction in the number of infections, days of antibiotic usage and hospitalisations during the 12 months of IVIg.…”

“…Patients with an IgG subclass deficiency or SADNI who suffer from severe or recurrent RTIs despite prophylactic antibiotics are candidates for Ig therapy [66,67].…”

An Update on the Use of Immunoglobulin for the Treatment of Immunodeficiency Disorders

“…A clear exception is the case of IgA deficiency with IgG2 subclass deficiency, as the loss of anti-carbohydrate antibody in such cases can lead to devastating bacterial illnesses and chronic lung disease [41,42]. Aside from these cases, there are few reports on the efficacy of IVIG in IgG subclass deficiency, although an open - label study evaluating efficacy of monthly replacement (albeit in a very small group of patients) showed deceased number of infections, decreased antibiotics, decreased hospitalizations and improved quality of life [43]. …”