Immunoglobulin A Deficiency Medication

Updated: May 15, 2018
  • Author: Marina Y Dolina, MD; Chief Editor: Michael A Kaliner, MD  more...
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Medication

Medication Summary

Immunoglobulin A deficiency (IgAD) has no specific treatment. Replacement therapy is not practical for IgAD because of the short half-life of IgA and the relative paucity of IgA in commercial immunoglobulin preparations.

Antibiotic therapy is the first line of treatment, specific for sinopulmonary or GI tract infections. Associated sinopulmonary infections are treated according to treatment protocols used for community-acquired respiratory tract infections in healthy persons, but prolonged treatment may be necessary.

Immunization with pneumococcal and other polysaccharide vaccines is important; however, not all patients are able to mount an immune response. Postvaccination IgG titers can be obtained to confirm the presence of an age-appropriate protective level of antipneumococcal IgG. Patients with common variable immunodeficiency (CVID) or more subtle specific antibody deficiencies may be unable to mount a response to polysaccharide antigens; therefore, pneumococcal vaccination in CVID patients is often ineffective.

Use of IGIV as replacement therapy is not indicated for selective IgAD per se. In selected circumstances in patients with concomitant SIgAD and selective IgG antibody deficiency who have recurrent or chronic high-grade sinopulmonary infections, a trial of IGIV may be given to see if a substantial clinical response occurs. Most patients with IgAD as part of CVID and/or with concomitant specific IgG antibody deficiency can safely receive intravenous (IV) or subcutaneous (SC) IgG replacement therapy. [36, 81]

Patients with known or possible anti-IgA antibodies are still at increased risk of anaphylaxis or severe IgG-mediated reactions.

Precautions must be used in the administration of IV immunoglobulin and other blood products in patients with IgAD because IV immunoglobulin preparations and other blood products contain at least small amounts of IgA.

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Vaccines, inactivated bacteria

Class Summary

Used to induce active immunity.

Pneumococcal vaccine polyvalent (PPV23; Pneumovax 23; Pnu-Imune 23)

Contains capsular polysaccharides of 23 pneumococcal types, which comprise 98% of pneumococcal disease isolates. For use in children >2 y and adults at increased risk of pneumococcal disease and its complications because of other underlying health conditions. Also benefits adults ≥ 65 y.

Pneumococcal vaccine heptavalent (PCV7; Prevnar)

Pneumococcal conjugate vaccine approved for infants and toddlers. Contains 7 purified capsular polysaccharides of S pneumoniae serotypes, accounting for 71% of infection among children < 24 m, each coupled with a nontoxic variant of diphtheria toxin, CRM 197.

Licensed for use in infants and young children in Feb 2000. Recommended for children aged 2-23 mo and for children aged 24-59 mo who are at increased risk for pneumococcal disease (eg, with sickle cell disease, HIV infection, other immunocompromising or chronic medical conditions). Licensed for infants aged ≥ 6 wk.

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