IgA and IgG Subclass Deficiencies Follow-up

Updated: Aug 14, 2014
  • Author: Terry W Chin, MD, PhD; Chief Editor: Harumi Jyonouchi, MD  more...
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Follow-up

Further Outpatient Care

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  • Because immunoglobulin (Ig)G subclass and/or isolated IgA deficiency are observed most commonly in atopic or autoimmune disorders, treating the concomitant diseases is crucial. Adequate treatment for the underlying allergic component may substantially decrease the rate of chronic upper and lower respiratory infections.

  • Live viral vaccines are not a risk in partial defects. When the diagnosis of partial defects is established, live viral vaccines can be administered. However, the risk occurs when the exact diagnosis is not known. Until concomitant T-cell deficiency is ruled out, live viral vaccines should be avoided in the patient and in siblings or other children in the house because the attenuated virus is excreted and poses a threat to the severely immunodeficient patient.

  • Sorensen et al (1998) showed that a significant percentage of children with specific antibody deficiency develop protective antibody levels to the conjugated pneumococcal vaccine (Prevnar) with subsequent decreased infections. [60]

  • In addition, serum IgA subclass levels, IgG subclass levels, or both may increase to normal range in more than 50%. [50] Therefore, regular monitoring of immunoglobulin levels should be done. Conversely, levels may decrease and lose specific immunoglobulin production as some patients progress to common variable immunodeficiency (CVID).

  • Do not make a decision to start IVIg treatment lightly. Although its beneficial effect for patients with agammaglobulinemia or hypogammaglobulinemia is not controversial, its use in IgG subclass deficiency or specific antibody deficiency in the presence of normal total Igs is not routine. In 1999, Lawton pointed out that patients, their parents, or the referring physician often insist on a trial of Ig therapy. [61] When this results in a beneficial effect, discontinuation is often difficult because "the child's health and family peace soon become dependent on continuation of the treatment." [61]

  • In addition to the high cost of IVIg and the emotional stress of monthly infusions, keep in mind the real possibility of IVIg transmitting unknown infectious agents. Hepatitis C virus transmission has been documented with certain IVIg preparations.

  • Families usually agree to a predetermined interval for an empiric trial of IVIg if the clinical problems with recurrent upper and lower respiratory infections appear to be sufficiently severe in patients younger than 4 years. This may occur during the winter, ending in spring. Immunologic testing then can be repeated 3-4 months after the last infusion. Restart treatment only if the antibody response remains abnormal.

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Complications

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  • Prevent chronic upper and lower respiratory disorders by treating underlying allergic disorders (eg, rhinitis, asthma) with aggressive use of antibiotics to manage bacterial infections (eg, sinusitis, bronchitis, pneumonias). Chronic ear infections can cause hearing loss. Watch for mastoiditis.

  • In one study, chronic pulmonary damage, as determined on chest CT scanning, occurred in about 20% of patients. [49] In the converse, extensive evaluation of patients with bronchiectasis showed that 11% had polysaccharide antibody deficiency to either pneumococcal or HIB vaccines. [29]

  • No good studies have been conducted to examine the potential benefits of prophylactic antibiotics, given systemically or topically (ie, aerosolized), in patients with B-cell disorder. However, it can be invaluable in selected patients.

  • Patients with impaired responses to vaccines may eventually develop CVID, possibly with concomitant T-cell deficiency. Closely follow up such patients and serially assess specific antibody responses (protein and polysaccharide antigens) and IgG subclasses and/or IgA serum levels.

  • Some IgA deficiency also occurs in patients with T lymphocyte deficiency such as ataxia-telangiectasia. Live viral vaccines should be avoided in these patients.

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Prognosis

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  • The prognosis of patients with these B-cell disorders depends on the severity of the humoral deficiency and on the extent to which other associated disorders, such as atopic or autoimmune diseases, are involved.

  • IVIg therapy offers a good prognosis for patients with the most severe form of antibody deficiency (ie, agammaglobulinemia).

  • In many individuals, selective IgA deficiency occurs without respiratory or intestinal symptoms. The role of selective IgG deficiency role in susceptibility to infections is similarly uncertain because many individuals with selective IgG deficiency are asymptomatic.

  • Most patients with IgA and IgG subclass deficiency who are younger than 4 years do well with appropriate antibiotic therapy and outgrow their frequent or recurrent infections, especially if they can make specific antibodies. Closely monitor patients who have IgG subclass deficiency that does not improve to assess for relatively complete B-cell deficiency such as CVID. [10]

  • Wolpert and Knutsen reported on 120 children with specific antibody deficiency and found that 50% developed normal responses after a mean of 3.1 years. [62] IVIg was administered in 28% of these children. Similar findings were reported by Kutukculer et al. [50]

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Patient Education

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  • Because of the close association with various allergic and autoimmune disorders, parents and patients must be aware of any underlying chronic atopic disorders.

  • Possible allergic triggers may require additional intervention with allergy testing and medical treatment.

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